(Q28555118)

1

0 references

Nicole Scott-Hewitt

2

0 references

Christoph Pröschel

3

0 references

Margot Mayer-Pröschel

4

0 references

Mark Noble

Creative Commons Attribution 4.0 International

5

0 references

language of work or name

English

0 references

publication date

December 2016

0 references

published in

PLOS Biology

0 references

volume

14

0 references

issue

12

0 references

page(s)

e1002583

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copyright license

start time

15 December 2016

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

0 references

cites work

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Impairment of homeostasis in lysosomal storage disorders

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The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunction

19 March 2017

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Protective effects of positive lysosomal modulation in Alzheimer's disease transgenic mouse models

19 March 2017

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Differential neuroprotective and antiinflammatory effects of estrogen receptor (ER)alpha and ERbeta ligand treatment

19 March 2017

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Psychosine-induced apoptosis and cytokine activation in immune peripheral cells of Krabbe patients

19 March 2017

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Single-dose intracerebroventricular administration of galactocerebrosidase improves survival in a mouse model of globoid cell leukodystrophy

19 March 2017

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Lysophospholipid receptors: signalling, pharmacology and regulation by lysophospholipid metabolism

19 March 2017

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Discovery of pyrrolo[2,3-b]pyrazines derivatives as submicromolar affinity activators of wild type, G551D, and F508del cystic fibrosis transmembrane conductance regulator chloride channels

19 March 2017

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ClC-7 requires Ostm1 as a beta-subunit to support bone resorption and lysosomal function

19 March 2017

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TDAG8 is a proton-sensing and psychosine-sensitive G-protein-coupled receptor

19 March 2017

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Quantification of galactosylsphingosine in the twitcher mouse using electrospray ionization-tandem mass spectrometry

19 March 2017

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

19 March 2017

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Sphingosine-induced apoptosis is dependent on lysosomal proteases

19 March 2017

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Identification of a molecular target of psychosine and its role in globoid cell formation

19 March 2017

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The role of autophagy in neurodegenerative disease

19 March 2017

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Regulation of cell death: the calcium-apoptosis link

7 April 2017

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Should states adopt newborn screening for early infantile Krabbe disease?

7 April 2017

1 reference

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Nanoparticles restore lysosomal acidification defects: Implications for Parkinson and other lysosomal-related diseases

29 September 2017

1 reference

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Recent advances in gene therapy for lysosomal storage disorders

29 September 2017

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Neuronopathic lysosomal storage disorders: Approaches to treat the central nervous system

29 September 2017

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Mechanism-based combination treatment dramatically increases therapeutic efficacy in murine globoid cell leukodystrophy

29 September 2017

1 reference

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Mechanism of neuromuscular dysfunction in Krabbe disease.

29 September 2017

1 reference

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Lysosomal storage diseases: from pathophysiology to therapy.

29 September 2017

1 reference

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Role of extracellular calcium and mitochondrial oxygen species in psychosine-induced oligodendrocyte cell death

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Experimental therapies in the murine model of globoid cell leukodystrophy.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Lysosomotropic agents: impact on lysosomal membrane permeabilization and cell death

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Approaches for detecting lysosomal alkalinization and impaired degradation in fresh and cultured RPE cells: evidence for a role in retinal degenerations

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Sphingolipid lysosomal storage disorders

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Correction of lysosomal dysfunction as a therapeutic strategy for neurodegenerative diseases

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Treatment of lysosomal storage disorders: successes and challenges

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

The voltage-gated sodium channel TPC1 confers endolysosomal excitability.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Rescue of compromised lysosomes enhances degradation of photoreceptor outer segments and reduces lipofuscin-like autofluorescence in retinal pigmented epithelial cells

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Glucopsychosine increases cytosolic calcium to induce calpain-mediated apoptosis of acute myeloid leukemia cells

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Early axonal loss accompanied by impaired endocytosis, abnormal axonal transport, and decreased microtubule stability occur in the model of Krabbe's disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Therapeutic benefit of lentiviral-mediated neonatal intracerebral gene therapy in a mouse model of globoid cell leukodystrophy

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Treatment of lysosomal storage diseases: recent patents and future strategies.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Lysosomal alkalization and dysfunction in human fibroblasts with the Alzheimer's disease-linked presenilin 1 A246E mutation can be reversed with cAMP

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Neuronal inclusions of α-synuclein contribute to the pathogenesis of Krabbe disease.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Oral small molecule therapy for lysosomal storage diseases

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Clinical studies in lysosomal storage diseases: past, present and future

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Inhibition of angiogenesis by β-galactosylceramidase deficiency in globoid cell leukodystrophy.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Neuronopathic lysosomal storage diseases: clinical and pathologic findings.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

The sphingolipid psychosine inhibits fast axonal transport in Krabbe disease by activation of GSK3β and deregulation of molecular motors

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Glucosylceramide modulates endolysosomal pH in Gaucher disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Lysosomal leukodystrophies: Krabbe disease and metachromatic leukodystrophy.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Estrogen receptor β ligand therapy activates PI3K/Akt/mTOR signaling in oligodendrocytes and promotes remyelination in a mouse model of multiple sclerosis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

CD133/CD140a-based isolation of distinct human multipotent neural progenitor cells and oligodendrocyte progenitor cells

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Role of endogenous psychosine accumulation in oligodendrocyte differentiation and survival: implication for Krabbe disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

MMP-3 mediates psychosine-induced globoid cell formation: implications for leukodystrophy pathology

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Enzyme replacement therapy for lysosomal storage diseases

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Lysosome-targeted octadecyl-rhodamine B-liposomes enhance lysosomal accumulation of glucocerebrosidase in Gaucher's cells in vitro

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

New strategies for the treatment of lysosomal storage diseases (review).

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Inhibition of glycogen synthase kinase-3 ameliorates β-amyloid pathology and restores lysosomal acidification and mammalian target of rapamycin activity in the Alzheimer disease mouse model: in vivo and in vitro studies

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Beneficial effects of lysosome-modulating and other pharmacological and nanocarrier agents on amyloid-beta-treated cells

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Glucocerebrosidase deficiency in substantia nigra of parkinson disease brains

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Treatment options for lysosomal storage disorders: developing insights

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Enzyme replacement therapy for lysosomal diseases: lessons from 20 years of experience and remaining challenges

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Lysosome-dependent pathways as a unifying theme in Parkinson's disease.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Drosophila TRPML is required for TORC1 activation

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Bone marrow transplantation increases efficacy of central nervous system-directed enzyme replacement therapy in the murine model of globoid cell leukodystrophy

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Stimulation of the D5 dopamine receptor acidifies the lysosomal pH of retinal pigmented epithelial cells and decreases accumulation of autofluorescent photoreceptor debris

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Endolysosome involvement in LDL cholesterol-induced Alzheimer's disease-like pathology in primary cultured neurons

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Cystic fibrosis transmembrane conductance regulator contributes to reacidification of alkalinized lysosomes in RPE cells

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Positive lysosomal modulation as a unique strategy to treat age-related protein accumulation diseases

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Intracellular accumulation of aggregated pyroglutamate amyloid beta: convergence of aging and Aβ pathology at the lysosome

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Psychosine induces the dephosphorylation of neurofilaments by deregulation of PP1 and PP2A phosphatases

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Loss of lysosomal ion channel transient receptor potential channel mucolipin-1 (TRPML1) leads to cathepsin B-dependent apoptosis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Effect of psychosine on inducible nitric-oxide synthase expression under different culture conditions: implications for Krabbe disease.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

The cellular pathology of lysosomal diseases

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Peripheral neuropathy in the Twitcher mouse involves the activation of axonal caspase 3.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Systemic delivery of bone marrow-derived mesenchymal stromal cells diminishes neuropathology in a mouse model of Krabbe's disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Proteomic identification of novel targets regulated by the mammalian target of rapamycin pathway during oligodendrocyte differentiation

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Effect of intrastriatal mesenchymal stromal cell injection on progression of a murine model of Krabbe disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Bone marrow transplantation augments the effect of brain- and spinal cord-directed adeno-associated virus 2/5 gene therapy by altering inflammation in the murine model of globoid-cell leukodystrophy

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Therapeutic effects of remediating autophagy failure in a mouse model of Alzheimer disease by enhancing lysosomal proteolysis.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Mesenchymal lineage stem cells have pronounced anti-inflammatory effects in the twitcher mouse model of Krabbe's disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Persistence of psychosine in brain lipid rafts is a limiting factor in the therapeutic recovery of a mouse model for Krabbe disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Endocannabinoids prevent β-amyloid-mediated lysosomal destabilization in cultured neurons

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Eliglustat tartrate, an orally active glucocerebroside synthase inhibitor for the potential treatment of Gaucher disease and other lysosomal storage diseases.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Proteases and proteolysis in Alzheimer disease: a multifactorial view on the disease process

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Sensors and regulators of intracellular pH.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Disease-causing mutations in the cystic fibrosis transmembrane conductance regulator determine the functional responses of alveolar macrophages

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Akt signals through the mammalian target of rapamycin pathway to regulate CNS myelination.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Psychosine accumulates in membrane microdomains in the brain of krabbe patients, disrupting the raft architecture.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Revisiting the role of cystic fibrosis transmembrane conductance regulator and counterion permeability in the pH regulation of endocytic organelles

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Significant correction of pathology in brains of twitcher mice following injection of genetically modified mouse neural progenitor cells

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Unimpaired lysosomal acidification in respiratory epithelial cells in cystic fibrosis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Lysosulfatide regulates the motility of a neural precursor cell line via calcium-mediated process collapse

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Psychosine-induced alterations in peroxisomes of twitcher mouse liver

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

The role of AMPK in psychosine mediated effects on oligodendrocytes and astrocytes: implication for Krabbe disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Substrate deprivation therapy: a new hope for patients suffering from neuronopathic forms of inherited lysosomal storage diseases

29 September 2017

1 reference

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The role of calcium and other ions in sorting and delivery in the late endocytic pathway

29 September 2017

1 reference

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The role of lysosphingolipids in the regulation of biological processes

29 September 2017

1 reference

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Lysosomal cysteine cathepsins: signaling pathways in apoptosis

29 September 2017

1 reference

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Pathogenic mechanisms in lysosomal disease: a reappraisal of the role of the lysosome

29 September 2017

1 reference

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Chemically diverse toxicants converge on Fyn and c-Cbl to disrupt precursor cell function

29 September 2017

1 reference

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Krabbe disease: psychosine-mediated activation of phospholipase A2 in oligodendrocyte cell death

29 September 2017

1 reference

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Cathepsin-regulated apoptosis

29 September 2017

1 reference

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Proton-sensing and lysolipid-sensitive G-protein-coupled receptors: a novel type of multi-functional receptors

29 September 2017

1 reference

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Peroxisomal participation in psychosine-mediated toxicity: implications for Krabbe's disease

29 September 2017

1 reference

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Treatment of lysosomal storage disorders: cell therapy and gene therapy

29 September 2017

1 reference

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Psychosine-induced apoptosis in a mouse oligodendrocyte progenitor cell line is mediated by caspase activation

29 September 2017

1 reference

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New therapeutics for the treatment of glycosphingolipid lysosomal storage diseases

29 September 2017

1 reference

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Toxicity of glucosylsphingosine (glucopsychosine) to cultured neuronal cells: a model system for assessing neuronal damage in Gaucher disease type 2 and 3.

29 September 2017

1 reference

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Globoid Cell Leukodystrophy (Krabbe's Disease): Update

29 September 2017

1 reference

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Molecular mechanism of psychosine-induced cell death in human oligodendrocyte cell line

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion.

29 September 2017

1 reference

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Effectiveness of enzyme replacement therapy in 1028 patients with type 1 Gaucher disease after 2 to 5 years of treatment: a report from the Gaucher Registry

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Lysophospholipids and their G protein-coupled receptors in inflammation and immunity

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Galactosylsphingosine (psychosine)-induced expression of cytokine-mediated inducible nitric oxide synthases via AP-1 and C/EBP: implications for Krabbe disease

29 September 2017

1 reference

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Enzyme replacement therapy in Fabry disease: a randomized controlled trial

29 September 2017

1 reference

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Psychosine, cytokinesis, and orphan receptors. Unexpected connections

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Lysosomal membrane damage in soluble Abeta-mediated cell death in Alzheimer's disease

29 September 2017

1 reference

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Migration assays for oligodendrocyte precursor cells

29 September 2017

1 reference

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Inhibition of cytokinesis by a lipid metabolite, psychosine

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Lipid changes in Niemann-Pick disease type C brain: personal experience and review of the literature

29 September 2017

1 reference

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Twenty five years of the "psychosine hypothesis": a personal perspective of its history and present status

29 September 2017

1 reference

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In vitro accumulation of glucocerebroside in neuroblastoma cells: a model for study of Gaucher disease pathobiology

29 September 2017

1 reference

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Ca2+ mobilizing action of sphingosine in Jurkat human leukemia T cells. Evidence that sphingosine releases Ca2+ from inositol trisphosphate- and phosphatidic acid-sensitive intracellular stores through a mechanism independent of inositol trisphospha

29 September 2017

1 reference

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Gangliosides and glycosphingolipids as modulators of cell growth, adhesion, and transmembrane signaling.

29 September 2017

1 reference

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Effects of psychosine (galactosylsphingosine) on the survival and the fine structure of cultured Schwann cells

29 September 2017

1 reference

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Effect of weak bases on the intralysosomal pH in mouse peritoneal macrophages

29 September 2017

1 reference

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Globoid cell leucodystrophy (Krabbe's disease): deficiency of galactocerebroside beta-galactosidase

29 September 2017

1 reference

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Additional deficiency of psychosine galactosidase in globoid cell leukodystrophy: an implication to enzyme replacement therapy.

29 September 2017

1 reference

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Lysosomal storage diseases: heterogeneous group of disorders

29 September 2017

1 reference

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Neuronal accumulation of glucosylceramide in a mouse model of neuronopathic Gaucher disease leads to neurodegeneration

3 June 2018

1 reference

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Oxidative stress as a therapeutic target in globoid cell leukodystrophy.

3 June 2018

1 reference

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Lipid storage disorders block lysosomal trafficking by inhibiting a TRP channel and lysosomal calcium release

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Lysosomal pH and analysis of the counter ion pathways that support acidification

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

A cation counterflux supports lysosomal acidification

3 June 2018

1 reference

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Increased activity and altered subcellular distribution of lysosomal enzymes determine neuronal vulnerability in Niemann-Pick type C1-deficient mice

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration

3 June 2018

1 reference

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Cytokinesis inhibition by glycosphingolipid, psychosine

3 June 2018

1 reference

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Sphingolipid derivatives modulate intracellular Ca2+ in rat synaptosomes

3 June 2018

1 reference

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Stimulation of adenylyl cyclase and induction of brain-derived neurotrophic factor and TrkB mRNA by NKH477, a novel and potent forskolin derivative.

3 June 2018

1 reference

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Responses to cyclic AMP is impaired in the twitcher Schwann cells in vitro.

3 June 2018

1 reference

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A role in migration for the alpha V beta 1 integrin expressed on oligodendrocyte precursors.

3 June 2018

1 reference

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Accumulation of tissue glucosylsphingosine in Gaucher-like mouse induced by the glucosylceramidase inhibitor cyclophellitol.

3 June 2018

1 reference

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Effects of sphingosine derivatives on MC3T3-E1 pre-osteoblasts: psychosine elicits release of calcium from intracellualr stores

3 June 2018

1 reference

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Accumulation of glucosylceramide and glucosylsphingosine (psychosine) in cerebrum and cerebellum in infantile and juvenile Gaucher disease

3 June 2018

1 reference

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Progressive accumulation of toxic metabolite in a genetic leukodystrophy

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Analysis of galactosylsphingosine (psychosine) in the brain.

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Globoid cell leukodystrophy: Additional deficiency of psychosine galactosidase

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Glycosphingolipid studies of visceral tissues and brain from type 1 Gaucher disease variants

3 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5169359

Cerebrosides and psychosine disrupt mitochondrial functions.

3 June 2018

1 reference

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Accumulation of galactosylsphingosine (psychosine) in the twitcher mouse: determination by HPLC.

3 June 2018

1 reference

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Globoid cell leukodystrophy is a generalized galactosylsphingosine (psychosine) storage disease

3 June 2018

1 reference

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Lysosphingolipids inhibit protein kinase C: implications for the sphingolipidoses

3 June 2018

1 reference

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Accumulation of lysosulfatide (sulfogalactosylsphingosine) in tissues of a boy with metachromatic leukodystrophy

3 June 2018

1 reference

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Effects of various lysosphingolipids on cell growth, morphology and lipid composition in three neuroblastoma cell lines

3 June 2018

1 reference

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Evaluation of synthetic sphingosine, lysosphingolipids and glycosphingolipids as inhibitors of functional responses of human neutrophils

3 June 2018

1 reference

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Novel procedure for measuring psychosine derivatives by an HPLC method.

3 June 2018

1 reference

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Chemical pathology of Krabbe's disease. III. Ceramide-hexosides and gangliosides of brain

3 June 2018

1 reference

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Chemical pathology of Krabbe disease: the occurrence of psychosine and other neutral sphingoglycolipids

3 June 2018

1 reference

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Central nervous system-directed AAV2/5-mediated gene therapy synergizes with bone marrow transplantation in the murine model of globoid-cell leukodystrophy

3 June 2018

1 reference

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Identification of hematopoietic stem cell-specific miRNAs enables gene therapy of globoid cell leukodystrophy

21 January 2018

1 reference

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Primary bone marrow mesenchymal stromal cells rescue the axonal phenotype of Twitcher mice

21 January 2018

1 reference

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Widespread enzymatic correction of CNS tissues by a single intracerebral injection of therapeutic lentiviral vector in leukodystrophy mouse models

21 January 2018

1 reference

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Intraventricular administration of recombinant adenovirus to neonatal twitcher mouse leads to clinicopathological improvements

21 January 2018

1 reference

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Widespread and highly persistent gene transfer to the CNS by retrovirus vector in utero: implication for gene therapy to Krabbe disease

21 January 2018

1 reference

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CNS-targeted AAV5 gene transfer results in global dispersal of vector and prevention of morphological and function deterioration in CNS of globoid cell leukodystrophy mouse model

21 January 2018

1 reference

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AAV-mediated expression of galactocerebrosidase in brain results in attenuated symptoms and extended life span in murine models of globoid cell leukodystrophy

21 January 2018

1 reference

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Enzyme replacement therapy improves peripheral nerve and sweat function in Fabry disease.

21 January 2018

1 reference

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Enzyme replacement therapy of a novel humanized mouse model of globoid cell leukodystrophy.

21 January 2018

1 reference

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Dysfunction of peroxisomes in twitcher mice brain: a possible mechanism of psychosine-induced disease

21 January 2018

1 reference

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The galactocerebrosidase enzyme contributes to maintain a functional neurogenic niche during early post-natal CNS development

21 January 2018

1 reference

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Insulin-like growth factor-1 provides protection against psychosine-induced apoptosis in cultured mouse oligodendrocyte progenitor cells using primarily the PI3K/Akt pathway

21 January 2018

1 reference

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Altered expression and distribution of cathepsins in neuronopathic forms of Gaucher disease and in other sphingolipidoses.

21 January 2018

1 reference

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IGF-1-stimulated protein synthesis in oligodendrocyte progenitors requires PI3K/mTOR/Akt and MEK/ERK pathways.

21 January 2018

1 reference

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The Cl-/H+ antiporter ClC-7 is the primary chloride permeation pathway in lysosomes.

21 January 2018

1 reference

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Lactosylceramide in lysosomal storage disorders: a comparative immunohistochemical and biochemical study

21 January 2018

1 reference

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Glucosylceramide modulates membrane traffic along the endocytic pathway

21 January 2018

1 reference

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Immunohistological study of globoid cell leukodystrophy.

21 January 2018

1 reference

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Axonopathy is a compounding factor in the pathogenesis of Krabbe disease.

21 January 2018

1 reference

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Lysosomal pathology and osteopetrosis upon loss of H+-driven lysosomal Cl- accumulation

21 January 2018

1 reference

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Differential Effects of Glycosphingolipids n Protein Kinase C Activity in PC12D Pheochromocytoma Cells

21 January 2018

1 reference

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Krabbe disease: involvement of connexin43 in the apoptotic effects of sphingolipid psychosine on mouse oligodendrocyte precursors.

21 January 2018

1 reference

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Galactosylsphingosine (psychosine)-induced demyelination is attenuated by sphingosine 1-phosphate signalling.

21 January 2018

1 reference

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Psychosine is as potent an inducer of cell death as C6-ceramide in cultured fibroblasts and in MOCH-1 cells

21 January 2018

1 reference

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Glucosylceramide and glucosylsphingosine modulate calcium mobilization from brain microsomes via different mechanisms

21 January 2018

1 reference

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Inhibition by sulfatide of 21-kDa protein phosphorylation by protein kinase C in cow mammary gland and its reversal by phosphatidylserine

21 January 2018

1 reference

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Lyso-glycosphingolipids mobilize calcium from brain microsomes via multiple mechanisms

21 January 2018

1 reference

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Psychosine inhibits osteoclastogenesis and bone resorption via G protein-coupled receptor 65.

21 January 2018

1 reference

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TNF compromises lysosome acidification and reduces α-synuclein degradation via autophagy in dopaminergic cells.

21 January 2018

1 reference

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Antioxidants are required during the early critical period, but not later, for neuronal survival

21 January 2018

1 reference

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Comprehensive quantitative analysis of bioactive sphingolipids by high-performance liquid chromatography-tandem mass spectrometry

21 January 2018

1 reference

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Newborn screening for Krabbe disease in New York State: the first eight years' experience.

21 January 2018

1 reference

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[Enzyme Replacement Therapy for Pompe Disease: The Long-Term Efficacy and Limitation].

27 November 2018

1 reference

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Apoptosis of Neuro2a cells induced by lysosphingolipids with naturally occurring stereochemical configurations.

27 November 2018

1 reference

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Effect of psychosine on mitochondrial function.

27 November 2018

1 reference

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Physicochemical characterization of psychosine by 1H nuclear magnetic resonance and electron microscopy

27 November 2018

1 reference

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Teaching old drugs new tricks. Meeting of the Neurodegeneration Drug Screening Consortium, 7-8 April 2002, Washington, DC, USA

27 November 2018

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12 December 2020

Treatment with an estrogen receptor alpha ligand is neuroprotective in experimental autoimmune encephalomyelitis

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Gene therapy for lysosomal storage disorders

1 reference

12 December 2020

Tissue culture model of Krabbe's disease: psychosine cytotoxicity in rat oligodendrocyte culture

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12 December 2020

Impairment of protein kinase C activity in twitcher Schwann cells in vitro

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12 December 2020

Glycolipids of the spinal cord, sciatic nerve, and systemic organs of the twitcher mouse

1 reference

12 December 2020

L-cycloserine slows the clinical and pathological course in mice with globoid cell leukodystrophy (twitcher mice)

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12 December 2020

Phosphorylation of myelin basic protein in intact oligodendrocytes: inhibition by galactosylsphingosine and cyclic AMP

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12 December 2020

Do sphingoid bases interact with the peroxisome proliferator activated receptor alpha (PPAR-alpha)?

1 reference

12 December 2020

Sphingosine and psychosine, suggested inhibitors of protein kinase C, inhibit LH effects in rat luteal cells

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12 December 2020

Bafilomycin A1 inhibits lysosomal, phagosomal, and plasma membrane H(+)-ATPase and induces lysosomal enzyme secretion in macrophages

1 reference

12 December 2020

Glucosylceramide and glucosylsphingosine metabolism in cultured fibroblasts deficient in acid beta-glucosidase activity

1 reference

12 December 2020

Increased TNF-alpha production by peripheral blood mononuclear cells in patients with Krabbe's disease: effect of psychosine

1 reference

12 December 2020

Lysosulfatide (sulfogalactosylsphingosine) accumulation in tissues from patients with metachromatic leukodystrophy

1 reference

12 December 2020

Activation of phosphatidylinositol-specific phospholipase C by HDL-associated lysosphingolipid. Involvement in mitogenesis but not in cholesterol efflux

1 reference

12 December 2020

CFTR regulates phagosome acidification in macrophages and alters bactericidal activity

1 reference

12 December 2020

Lysosomal hydrolases in cerebrospinal fluid from subjects with Parkinson's disease

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12 December 2020

Hereditary leucodystrophy in the mouse: the new mutant twitcher

1 reference

12 December 2020

Krabbe disease: a galactosylsphingosine (psychosine) lipidosis

1 reference

12 December 2020

Inhibition of cytochrome c oxidase and hemolysis caused by lysosphingolipids

1 reference

12 December 2020

Proliferative capacity of oligodendrocytes in the demyelinating twitcher spinal cord

1 reference

12 December 2020