(Q37744753)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20449662%20AND%20SRC:MED&resulttype=core&format=json

1 February 2020

title

Lessons learnt from animal models: pathophysiology of neuropathic lysosomal storage disorders (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20449662%20AND%20SRC:MED&resulttype=core&format=json

1 February 2020

1 reference

1

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1 February 2020

2

John J Hopwood

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20449662%20AND%20SRC:MED&resulttype=core&format=json

1 February 2020

publication date

7 May 2010

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20449662%20AND%20SRC:MED&resulttype=core&format=json

Journal of Inherited Metabolic Disease

1 February 2020

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20449662%20AND%20SRC:MED&resulttype=core&format=json

1 February 2020

volume

33

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20449662%20AND%20SRC:MED&resulttype=core&format=json

1 February 2020

issue

4

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20449662%20AND%20SRC:MED&resulttype=core&format=json

1 February 2020

page(s)

363-371

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20449662%20AND%20SRC:MED&resulttype=core&format=json

https://scigraph.springernature.com/pub.10.1007/s10545-010-9078-6

1 February 2020

exact match

0 references

cites work

Inhibition of leucocytic lysosomal enzymes by glycosaminoglycans in vitro

1 reference

https://api.crossref.org/works/10.1007%2FS10545-010-9078-6

Behavioral phenotyping of transgenic and knockout mice: practical concerns and potential pitfalls

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-010-9078-6

A critical evaluation of the ubiquitin-proteasome system in Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-010-9078-6

Gene therapy for lysosomal storage diseases (LSDs) in large animal models

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-010-9078-6

Cerebellar degeneration in the Niemann-Pick type C mouse.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-010-9078-6

NSAIDs increase survival in the Sandhoff disease mouse: synergy with N-butyldeoxynojirimycin

21 January 2018

1 reference

12 December 2020

Site-specific phosphorylation of tau accompanied by activation of mitogen-activated protein kinase (MAPK) in brains of Niemann-Pick type C mice

1 reference

12 December 2020

Enzyme replacement therapy from birth in a feline model of mucopolysaccharidosis type VI.

1 reference

12 December 2020

CNS-directed gene therapy for lysosomal storage diseases

1 reference

12 December 2020

Gene therapy for lysosomal storage disorders

1 reference

12 December 2020

N-acetylglucosamine 6-sulphatase deficiency in a Nubian goat: a model of Sanfilippo syndrome type D (mucopolysaccharidosis IIID)

1 reference

12 December 2020

Rapamycin and mTOR-independent autophagy inducers ameliorate toxicity of polyglutamine-expanded huntingtin and related proteinopathies

1 reference

12 December 2020

Altered gene expression in cells from patients with lysosomal storage disorders suggests impairment of the ubiquitin pathway.

1 reference

12 December 2020

Delivery of recombinant proteins via the cerebrospinal fluid as a therapy option for neurodegenerative lysosomal storage diseases.

1 reference

12 December 2020

Cathepsin D expression level affects alpha-synuclein processing, aggregation, and toxicity in vivo.

1 reference

12 December 2020

A model of mucopolysaccharidosis IIIB (Sanfilippo syndrome type IIIB): N‐acetyl‐α‐D‐glucosaminidase deficiency in Schipperke dogs

1 reference

12 December 2020

Lysosomal dysfunction results in altered energy balance

1 reference

12 December 2020

Mucopolysaccharidosis IIIA (Sanfilippo syndrome) in a New Zealand Huntaway dog with ataxia

1 reference

12 December 2020

Expression of immune-related molecules is downregulated in twitcher mice following bone marrow transplantation

1 reference

12 December 2020

Caprine mucopolysaccharidosis-IIID: clinical, biochemical, morphological and immunohistochemical characteristics.

1 reference

12 December 2020

Mechanism of abnormal growth in astrocytes derived from a mouse model of GM2 gangliosidosis.

1 reference

12 December 2020

The role of the substantia nigra in cognitive activity in cats

1 reference

12 December 2020

Initial sequence and comparative analysis of the cat genome

1 reference

12 December 2020

Functional correction of CNS lesions in an MPS-IIIA mouse model by intracerebral AAV-mediated delivery of sulfamidase and SUMF1 genes

1 reference

12 December 2020

Screening for the Mucopolysaccharidosis-IlIA gene in Huntaway dogs

1 reference

12 December 2020

Mannitol-facilitated CNS entry of rAAV2 vector significantly delayed the neurological disease progression in MPS IIIB mice

1 reference

12 December 2020

beta-Mannosidosis in a Salers calf: a new storage disease of cattle

1 reference

12 December 2020

GAP43 overexpression and enhanced neurite outgrowth in mucopolysaccharidosis type IIIB cortical neuron cultures.

1 reference

12 December 2020

A whole-genome assembly of the domestic cow, Bos taurus

1 reference

12 December 2020

Alpha-L-iduronidase Deficiency in a Cat: A Model of Mucopolysaccharidosis I

1 reference

12 December 2020

Treating lysosomal storage disorders: current practice and future prospects

1 reference

12 December 2020

Promising CNS-directed enzyme replacement therapy for lysosomal storage diseases

1 reference

12 December 2020

Developmental analysis of CNS pathology in the lysosomal storage disease alpha-mannosidosis

1 reference

12 December 2020

Significantly increased lifespan and improved behavioral performances by rAAV gene delivery in adult mucopolysaccharidosis IIIB mice

1 reference

12 December 2020

Neurodegeneration in the Niemann-Pick C mouse: glial involvement

1 reference

12 December 2020

Biochemical basis of type AB GM2 gangliosidosis in a Japanese spaniel

1 reference

12 December 2020

Differential susceptibility to striatal neurodegeneration induced by quinolinic acid and kainate in inbred, outbred and hybrid mouse strains

1 reference

12 December 2020

Canine models for human genetic neurodegenerative diseases

1 reference

12 December 2020

Long-lasting memories of obstacles guide leg movements in the walking cat.

1 reference

12 December 2020

Enhanced degradation of synaptophysin by the proteasome in mucopolysaccharidosis type IIIB.

1 reference

12 December 2020

Enzyme replacement therapy in a feline model of Maroteaux-Lamy syndrome

1 reference

12 December 2020

A mouse model for mucopolysaccharidosis type III A (Sanfilippo syndrome).

1 reference

12 December 2020

Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism

1 reference

12 December 2020

Effect of cisternal sulfamidase delivery in MPS IIIA Huntaway dogs--a proof of principle study.

1 reference

12 December 2020

Neuronal GM 1 Gangliosidosis in a Siamese Cat with β-Galactosidase Deficiency

1 reference

12 December 2020

Examination of intravenous and intra-CSF protein delivery for treatment of neurological disease.

1 reference

12 December 2020

Phenotype of arylsulfatase A-deficient mice: relationship to human metachromatic leukodystrophy

1 reference

12 December 2020

Neuronal accumulation of alpha- and beta-synucleins in the brain of a GM2 gangliosidosis mouse model

1 reference

12 December 2020

Sanfilippo syndrome: a mini-review

1 reference

12 December 2020

Mechanisms of neurodegeneration in mucopolysaccharidoses II and IIIB: analysis of human brain tissue

1 reference

12 December 2020

Clinical, neurophysiological, biochemical and morphological features of eyes in Persian cats with mannosidosis

1 reference

12 December 2020

Relationship between reluctance to eat novel foods and open-field behavior in sheep.

1 reference

12 December 2020

A novel missense mutation in lysosomal sulfamidase is the basis of MPS III A in a spontaneous mouse mutant

1 reference

12 December 2020

Sulfamidase deficiency in a family of Dachshunds: a canine model of mucopolysaccharidosis IIIA (Sanfilippo A).

1 reference

12 December 2020

Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIB

1 reference

12 December 2020

Protective role of endogenous gangliosides for lysosomal pathology in a cellular model of synucleinopathies

1 reference

12 December 2020

Early neurodegeneration progresses independently of microglial activation by heparan sulfate in the brain of mucopolysaccharidosis IIIB mice

1 reference

12 December 2020

Paired helical filament tau (PHFtau) in Niemann-Pick type C disease is similar to PHFtau in Alzheimer's disease

1 reference

12 December 2020

Mouse gene knockout models for the CLN2 and CLN3 forms of ceroid lipofuscinosis

1 reference

12 December 2020

Dysmyelinogenesis in animal model of GM1 gangliosidosis

1 reference

12 December 2020

Pathogenic cascades in lysosomal disease-Why so complex?

1 reference

12 December 2020

Injury and strain-dependent dopaminergic neuronal degeneration in the substantia nigra of mice after axotomy or MPTP.

1 reference

12 December 2020

The neurobiology of brain and cognitive reserve: mental and physical activity as modulators of brain disorders

1 reference

12 December 2020

Neuronal ceroid lipofuscinosis in Merino sheep.

1 reference

12 December 2020

Development of motor deficits in a murine model of mucopolysaccharidosis type IIIA (MPS-IIIA).

1 reference

12 December 2020

Caprine beta-mannosidosis: development of glial and myelin abnormalities in optic nerve and corpus callosum.

1 reference

12 December 2020

Genetic background markedly influences vulnerability of the hippocampal neuronal organization in the "twitcher" mouse model of globoid cell leukodystrophy

1 reference

12 December 2020

Beneficial effects of anti-inflammatory therapy in a mouse model of Niemann-Pick disease type C1.

1 reference

12 December 2020

Gene therapy progress and prospects: gene therapy of lysosomal storage disorders

1 reference

12 December 2020

Canine GM2 gangliosidosis: morphological and biochemical analysis

1 reference

12 December 2020

Open field locomotor activity and anxiety-related behaviors in mucopolysaccharidosis type IIIA mice.

1 reference

12 December 2020

Central nervous system inflammation is a hallmark of pathogenesis in mouse models of GM1 and GM2 gangliosidosis

1 reference

12 December 2020

Differential subcellular localization of cholesterol, gangliosides, and glycosaminoglycans in murine models of mucopolysaccharide storage disorders

1 reference

12 December 2020

Intracerebral injection of sulfamidase delays neuropathology in murine MPS-IIIA.

1 reference

12 December 2020

Skin biopsy: a useful tool in the diagnosis of lysosomal storage diseases

1 reference

12 December 2020

The role of autophagy-lysosome pathway in neurodegeneration associated with Parkinson's disease

1 reference

12 December 2020

Effects of litter size, sex and periconceptional ewe nutrition on side preference and cognitive flexibility in the offspring

1 reference

12 December 2020

Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease

1 reference

12 December 2020

Pathology of Mucopolysaccharidosis IIIA in Huntaway Dogs

1 reference

12 December 2020

A block of autophagy in lysosomal storage disorders

1 reference

12 December 2020

Metabolic adaptations to interrupted glycosaminoglycan recycling.

1 reference

12 December 2020

Sanfilippo syndrome type B, a lysosomal storage disease, is also a tauopathy

1 reference

12 December 2020

Selective neurodegeneration in murine mucopolysaccharidosis VII is progressive and reversible.

1 reference

12 December 2020

A two-year study with fibrillar beta-amyloid (Abeta) immunization in aged canines: effects on cognitive function and brain Abeta.

1 reference

12 December 2020

The ubiquitin proteasome system in Huntington's disease and the spinocerebellar ataxias

1 reference

12 December 2020

Acetyl-L-carnitine and alpha-lipoic acid supplementation of aged beagle dogs improves learning in two landmark discrimination tests.

1 reference

12 December 2020

Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation

1 reference

12 December 2020

Genome sequence, comparative analysis and haplotype structure of the domestic dog

1 reference

12 December 2020

Late onset neurodegeneration in the Cln3-/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation.

1 reference

12 December 2020

Lysosomal accumulation of SCMAS (subunit c of mitochondrial ATP synthase) in neurons of the mouse model of mucopolysaccharidosis III B.

1 reference

12 December 2020

On whether the environmental enrichment may provide cognitive and brain reserves.

1 reference

12 December 2020

Substrate reduction therapy

1 reference

12 December 2020

Substrate reduction therapy for lysosomal storage diseases

1 reference

12 December 2020

Characterization of a C57BL/6 congenic mouse strain of mucopolysaccharidosis type IIIA.

1 reference

12 December 2020

Proteomic analysis of microglial contribution to mouse strain-dependent dopaminergic neurotoxicity.

1 reference

12 December 2020

Effect of high dose, repeated intra-cerebrospinal fluid injection of sulphamidase on neuropathology in mucopolysaccharidosis type IIIA mice.

1 reference

12 December 2020

Tau deletion exacerbates the phenotype of Niemann-Pick type C mice and implicates autophagy in pathogenesis.

1 reference

12 December 2020

Macroautophagy--a novel Beta-amyloid peptide-generating pathway activated in Alzheimer's disease

1 reference

12 December 2020

Enzyme-replacement therapy from birth delays the development of behavior and learning problems in mucopolysaccharidosis type IIIA mice

1 reference

12 December 2020

Mucopolysaccharidosis: secondarily induced abnormal distribution of lysosomal isoenzymes

1 reference

12 December 2020

Genetic variations and treatments that affect the lifespan of the NPC1 mouse

1 reference

12 December 2020

Sanfilippo A disease in the fetus--comparison with pre- and postnatal cases

1 reference

12 December 2020

10.1007/S10545-010-9078-6

Identifiers

DOI

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20449662%20AND%20SRC:MED&resulttype=core&format=json

1 February 2020

1 reference