(Q37950897)

25 August 2017

title

Invertebrate models of lysosomal storage disease: what have we learned so far? (English)

1 reference

lysosomal storage disease

25 August 2017

main subject

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Samantha Hindle

1

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Sean T. Sweeney

object named as

Sean T Sweeney

series ordinal

3

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author name string

Sarita Hebbar

series ordinal

2

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25 August 2017

publication date

25 October 2011

1 reference

Invertebrate Neuroscience

25 August 2017

published in

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25 August 2017

volume

11

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25 August 2017

issue

2

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25 August 2017

page(s)

59-71

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https://scigraph.springernature.com/pub.10.1007/s10158-011-0125-2

25 August 2017

exact match

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cites work

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Niemann-Pick C1 Like 1 (NPC1L1) is the intestinal phytosterol and cholesterol transporter and a key modulator of whole-body cholesterol homeostasis

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Aberrant lysosomal carbohydrate storage accompanies endocytic defects and neurodegeneration in Drosophila benchwarmer

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The optic lobe of Drosophila melanogaster. I. A Golgi analysis of wild-type structure

21 January 2018

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Mutations of a Drosophila NPC1 gene confer sterol and ecdysone metabolic defects

21 January 2018

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Oxidative stress in Niemann-Pick disease, type C

21 January 2018

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Isolation and structural analysis of three neutral glycosphingolipids from a mixed population of Caenorhabditis elegans (Nematoda:Rhabditida).

21 January 2018

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The pathogenesis of glycosphingolipid storage disorders

21 January 2018

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Niemann-Pick type C disease involves disrupted neurosteroidogenesis and responds to allopregnanolone

21 January 2018

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Biosynthesis of truncated N-linked oligosaccharides results from non-orthologous hexosaminidase-mediated mechanisms in nematodes, plants, and insects

21 January 2018

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The trp gene is essential for a light-activated Ca2+ channel in Drosophila photoreceptors.

21 January 2018

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Sphingolipid activator protein deficiency in a 16-week-old atypical Gaucher disease patient and his fetal sibling: biochemical signs of combined sphingolipidoses

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alpha-Glucosidase deficiency in generalized glycogenstorage disease (Pompe's disease).

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The Caenorhabditis elegans mucolipin-like gene cup-5 is essential for viability and regulates lysosomes in multiple cell types

21 January 2018

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Palmitoyl-protein thioesterase 1 deficiency in Drosophila melanogaster causes accumulation of abnormal storage material and reduced life span

21 January 2018

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A Drosophila model of the Niemann-Pick type C lysosome storage disease: dnpc1a is required for molting and sterol homeostasis

21 January 2018

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Drosophila Niemann-Pick type C-2 genes control sterol homeostasis and steroid biosynthesis: a model of human neurodegenerative disease

21 January 2018

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Clinical aspects of neuropathic lysosomal storage disorders

21 January 2018

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A genomic screen for modifiers of tauopathy identifies puromycin-sensitive aminopeptidase as an inhibitor of tau-induced neurodegeneration

21 January 2018

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The sphingolipid transporter spns2 functions in migration of zebrafish myocardial precursors

21 January 2018

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Towards understanding the neuronal ceroid lipofuscinoses.

21 January 2018

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An over-expression system for characterizing Ppt1 function in Drosophila

21 January 2018

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Genetic modifiers of degeneration in the cathepsin D deficient Drosophila model for neuronal ceroid lipofuscinosis

21 January 2018

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The cation channel mucolipin-1 is a bifunctional protein that facilitates membrane remodeling via its serine lipase domain

21 January 2018

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The Drosophila fused lobes gene encodes an N-acetylglucosaminidase involved in N-glycan processing

21 January 2018

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NCR-1 and NCR-2, the C. elegans homologs of the human Niemann-Pick type C1 disease protein, function upstream of DAF-9 in the dauer formation pathways

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Niemann-Pick type C mutations cause lipid traffic jam.

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene.

21 January 2018

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The beta-hexosaminidase story in Toronto: from enzyme structure to gene mutation.

21 January 2018

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Morphological alterations in neocortical and cerebellar GABAergic neurons in a canine model of juvenile Batten disease

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Prevalence of lysosomal storage disorders

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Molecular characterization of the Drosophila trp locus: a putative integral membrane protein required for phototransduction

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Cathepsin D-deficient Drosophila recapitulate the key features of neuronal ceroid lipofuscinoses

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Mutations in the novel membrane protein spinster interfere with programmed cell death and cause neural degeneration in Drosophila melanogaster

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Identification of HE1 as the second gene of Niemann-Pick C disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Marked differences in cholesterol synthesis between neurons and glial cells from postnatal rats.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Coding of two sphingolipid activator proteins (SAP-1 and SAP-2) by same genetic locus

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

The spinster homolog, two of hearts, is required for sphingosine 1-phosphate signaling in zebrafish.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Neuronal loss of Drosophila NPC1a causes cholesterol aggregation and age-progressive neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Identification and characterization of Caenorhabditis elegans palmitoyl protein thioesterase1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Juvenile neuronal ceroid-lipofuscinosis (Batten disease): a brief review and update

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Spinster is required for autophagic lysosome reformation and mTOR reactivation following starvation

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Neuro-ophthalmology of late-onset Tay-Sachs disease (LOTS).

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

ON ARRESTED CEREBRAL DEVELOPMENT, WITH SPECIAL REFERENCE TO ITS CORTICAL PATHOLOGY.1

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Phenotypic interactions of spinster with the genes encoding proteins for cell death control in Drosophila melanogaster.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Suppression of the cup-5 mucolipidosis type IV-related lysosomal dysfunction by the inactivation of an ABC transporter in C. elegans

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Targeting of NPC1 to late endosomes involves multiple signals, including one residing within the putative sterol-sensing domain

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

A block of autophagy in lysosomal storage disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

A mutation in the saposin A coding region of the prosaposin gene in an infant presenting as Krabbe disease: first report of saposin A deficiency in humans

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Mucolipidosis type IV is caused by mutations in a gene encoding a novel transient receptor potential channel

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Unrestricted synaptic growth in spinster-a late endosomal protein implicated in TGF-beta-mediated synaptic growth regulation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

A model for niemann-pick type C disease in the nematode Caenorhabditis elegans

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Rapamycin activation of 4E-BP prevents parkinsonian dopaminergic neuron loss.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Caenorhabditis elegans functional orthologue of human protein h-mucolipin-1 is required for lysosome biogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Naturally occurring mutations in GM2 gangliosidosis: a compendium

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Interactions between the juvenile Batten disease gene, CLN3, and the Notch and JNK signalling pathways.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

The Batten disease gene CLN3 is required for the response to oxidative stress.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Non-neuronopathic Gaucher disease due to saposin C deficiency.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Lipids of the Golgi membrane

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Lipid imbalance in the neurological disorder, Niemann-Pick C disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Motor deficit in a Drosophila model of mucolipidosis type IV due to defective clearance of apoptotic cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Common and uncommon pathogenic cascades in lysosomal storage diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Drosophila NPC1b promotes an early step in sterol absorption from the midgut epithelium

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Distribution of ectopic neurite growth and other geometrical distortions of CNS neurons in feline GM2 gangliosidosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Drosophila spichthyin inhibits BMP signaling and regulates synaptic growth and axonal microtubules

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

The cholesterol trafficking protein NPC1 is required for Drosophila spermatogenesis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

ER and oxidative stresses are common mediators of apoptosis in both neurodegenerative and non-neurodegenerative lysosomal storage disorders and are alleviated by chemical chaperones

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Spinster controls Dpp signaling during glial migration in the Drosophila eye.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Critical role for glycosphingolipids in Niemann-Pick disease type C.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Zwitterionic and acidic glycosphingolipids of the Drosophila melanogaster embryo

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10158-011-0125-2

Glycosphingolipid degradation and animal models of GM2-gangliosidoses

21 January 2018

1 reference

12 December 2020

Phenotype variations in Gaucher disease

1 reference

12 December 2020

The precursor of sulfatide activator protein is processed to three different proteins

1 reference

12 December 2020

Oxidative stress induces overgrowth of the Drosophila neuromuscular junction

1 reference

12 December 2020

Structure of full-length cDNA coding for sulfatide activator, a Co-beta-glucosidase and two other homologous proteins: two alternate forms of the sulfatide activator

1 reference

12 December 2020

Regulation of endocytosis by CUP-5, the Caenorhabditis elegans mucolipin-1 homolog

1 reference

12 December 2020

Storage solutions: treating lysosomal disorders of the brain

1 reference

12 December 2020

Genetic analysis of endocytosis in Caenorhabditis elegans: coelomocyte uptake defective mutants

1 reference

12 December 2020

Lysosomal storage diseases as disorders of autophagy

1 reference

12 December 2020

Glycosphingolipids in insects. Chemical structures of ceramide tetra-, penta-, hexa-, and heptasaccharides from Calliphora vicina pupae (Insecta: Diptera).

1 reference

12 December 2020

Modelling lysosomal storage disease in Drosophila.

1 reference

12 December 2020

Localization of the murine Niemann-Pick C1 protein to two distinct intracellular compartments

1 reference

12 December 2020

10.1007/S10158-011-0125-2

Identifiers

DOI

1 reference

25 August 2017

1 reference