ReviewPhilosophical transactions of the Royal Society of London. Series B, Biological sciences2003
Endocytic trafficking of glycosphingolipids in sphingolipid storage diseases.
Review in Philosophical transactions of the Royal Society of London. Series B, Biological sciences, 2003. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers.
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Who cites it
39 citing papers in PubMed, 77 citations in OpenAlex.
- Endosomes as central hubs of interorganellar communication and cellular homeostasis.Protein & cell · 2026Review
- Niemann-Pick C-like Endolysosomal Dysfunction in DHDDS Patient Cells, a Congenital Disorder of Glycosylation, Can Be Treated with Miglustat.International journal of molecular sciences · 2025Article
- Juvenile mucopolysaccharidosis plus disease caused by a missense mutation in VPS33A.Human mutation · 2022Article
- Neurodegeneration in Niemann-Pick Type C Disease: An Updated Review on Pharmacological and Non-Pharmacological Approaches to Counteract Brain and Cognitive Impairment.International journal of molecular sciences · 2021Review
- Community-Acquired Respiratory Distress Syndrome Toxin: Unique Exotoxin forFrontiers in microbiology · 2021Review
- Visualisation of cholesterol and ganglioside GM1 in zebrafish models of Niemann-Pick type C disease and Smith-Lemli-Opitz syndrome using light sheet microscopy.Histochemistry and cell biology · 2020Article
- LRRK2-Related Parkinson's Disease Due to Altered Endolysosomal Biology With Variable Lewy Body Pathology: A Hypothesis.Frontiers in neuroscience · 2020Review
- Sphingolipid abnormalities in cancer multidrug resistance: Chicken or egg?Cellular signalling · 2017Review
- Annexin A6-balanced late endosomal cholesterol controls influenza A replication and propagation.mBio · 2013Article
- Lysosomal dysfunction in a mouse model of Sandhoff disease leads to accumulation of ganglioside-bound amyloid-β peptide.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2012Article
- TRPML: transporters of metals in lysosomes essential for cell survival?Cell calcium · 2011Review
- Glycosphingolipid functions.Cold Spring Harbor perspectives in biology · 2011Review
- ARF6-mediated endocytic recycling impacts cell movement, cell division and lipid homeostasis.Seminars in cell & developmental biology · 2011Review
- Pathology and current treatment of neurodegenerative sphingolipidoses.Neuromolecular medicine · 2010Review
- Cholesterol pathways affected by small molecules that decrease sterol levels in Niemann-Pick type C mutant cells.PloS one · 2010Article
- Pathophysiology of neuropathic lysosomal storage disorders.Journal of inherited metabolic disease · 2010Review
- Sphingosine 1-phosphate lyase deficiency disrupts lipid homeostasis in liver.The Journal of biological chemistry · 2010Article
- Glycolipid trafficking in Drosophila undergoes pathway switching in response to aberrant cholesterol levels.Molecular biology of the cell · 2010Article
- Occurrence of an anomalous endocytic compartment in fibroblasts from Sandhoff disease patients.Molecular and cellular biochemistry · 2010Article
- Chemical screen to reduce sterol accumulation in Niemann-Pick C disease cells identifies novel lysosomal acid lipase inhibitors.Biochimica et biophysica acta · 2009Article
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Authors and funding
1 author at 1 institution in 1 country.
Funding
Abstract
In this review, recent studies of membrane lipid transport in sphingolipid (SL) storage disease (SLSD) fibroblasts are summarized. Several fluorescent glycosphingolipid (GSL) analogues are internalized from the plasma membrane via caveolae and are subsequently transported to the Golgi complex of normal fibroblasts, while in 10 different SLSD cell types, these lipids accumulate in endosomes and lysosomes. Additional studies have shown that cholesterol homeostasis is perturbed in multiple SLSDs secondary to accumulation of endogenous SLs, and that mis-targeting of the GSLs is regulated by cellular cholesterol. Golgi targeting of GSLs internalized via caveolae is dependent on microtubules and phosphoinositide 3-kinase(s) and is inhibited by expression of dominant-negative rab7 and rab9 constructs. Overexpression of wild-type rab7 or rab9 (but not rab11) in Niemann-Pick C fibroblasts results in correction of lipid trafficking defects, including restoration of Golgi targeting of fluorescent lactosylceramide and endogenous GM1 ganglioside (monitored by the transport of fluorescent cholera toxin), and a dramatic reduction in accumulation of intracellular cholesterol. These results suggest an approach for restoring normal lipid trafficking in this, and perhaps other, SLSD cell types, and may provide a basis for future therapy of these diseases.
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