ReviewJournal of neuroscience research2016
How membrane dysfunction influences neuronal survival pathways in sphingolipid storage disorders.
Review in Journal of neuroscience research, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
11 citing papers in PubMed, 16 citations in OpenAlex.
- Extracellular and Membrane Protein: Structure, Biological Functions, Diseases, and an Emerging Modality for Drug Discovery.MedComm · 2026Review
- Relevance of multilamellar and multicompartmental vesicles in biological fluids: understanding the significance of proportional variations and disease correlation.Biomarker research · 2023Review
- Identification of a DNA methylation signature for Renpenning syndrome (RENS1), a spliceopathy.European journal of human genetics : EJHG · 2023Article
- The long and the short of Huntington's disease: how the sphingolipid profile is shifted in the caudate of advanced clinical cases.Brain communications · 2022Article
- Elovl5 is required for proper action potential conduction along peripheral myelinated fibers.Glia · 2021Article
- Alteration of Sphingolipids in Biofluids: Implications for Neurodegenerative Diseases.International journal of molecular sciences · 2019Review
- Lipid Analysis of 30 000 Individual Rodent Cerebellar Cells Using High-Resolution Mass Spectrometry.Analytical chemistry · 2019Article
- Quantitative Microproteomics Based Characterization of the Central and Peripheral Nervous System of a Mouse Model of Krabbe Disease.Molecular & cellular proteomics : MCP · 2019Article
- Inhibition of the IGF-1-PI3K-Akt-mTORC2 pathway in lipid rafts increases neuronal vulnerability in a genetic lysosomal glycosphingolipidosis.Disease models & mechanisms · 2019Article
- Sphingolipids in neurodegeneration (with focus on ceramide and S1P).Advances in biological regulation · 2018Review
- Article
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
Sphingolipidoses are a class of inherited diseases that result from the toxic accumulation of undigested sphingolipids in lysosomes and other cellular membranes. Sphingolipids are particularly enriched in cells of the nervous system, and their excessive accumulation during disease has a significant impact on the nervous system. Neuronal dysfunction followed by neurological compromise is a common feature in many of these diseases; however, the underlying mechanisms that cause vulnerability of neurons are not fully understood. The plasma membrane plays a critical role in regulating cellular survival pathways, and its dysfunction has been implicated in neuronal failure in various adult-onset neuropathies. In the context of sphingolipidoses, we hypothesize that gradual accumulation of undigested lipids in plasma membranes causes local disruptions in lipid raft domains, leading to deregulation of multiple signaling pathways important for neuronal survival and function. We propose that defects in downstream signaling as a result of membrane dysfunction are common mechanisms underlying neuronal vulnerability in sphingolipid storage disorders with neurological compromise. © 2016 Wiley Periodicals, Inc.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.