ReviewLipids in health and disease2021
Sphingolipid lysosomal storage diseases: from bench to bedside.
Review in Lipids in health and disease, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers.
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Who cites it
37 citing papers in PubMed.
- Spatial Lipidomic Profiling of Kidney Functional Units in SIV-Infected and ART-Treated Rhesus Macaques.ACS measurement science au · 2026Article
- Beyond One-Size-Fits-All: Individually Tailored Dietary Interventions in Modern Obesity Care.Nutrients · 2026Review
- Contradictory Effects on Hepatocytes in ASMD.International journal of molecular sciences · 2026Review
- Sphingolipidomic Profiling of Peripheral Blood Mononuclear Cells Reveals a Distinct Immunometabolic Signature Across Patients with Essential Obesity and Metabolic Syndrome Compared to Normal-Weight Healthy Subjects.Journal of clinical medicine · 2026Article
- Sphingolipidoses: expanding the spectrum of α-synucleinopathies.Journal of neural transmission (Vienna, Austria : 1996) · 2026Review
- Transport of sphingolipids by yeast Npc2 supports phase separation of the vacuole membrane.The Journal of biological chemistry · 2026Article
- Advances in pH-Sensitive Luminescent Transition Metal Complexes for Theranostic Applications.Chemistry, an Asian journal · 2026Review
- Unlocking the therapeutic potential of sphingolipids in wound healing: from molecular mechanisms to clinical applications.International journal of surgery (London, England) · 2026Review
- The physiological and pathological effects of sphingolipid metabolism and signaling in the central nervous system.Brain pathology (Zurich, Switzerland) · 2026Review
- From "lysosomal addiction" to targeted therapies: exploiting novel windows in colorectal cancer.European journal of medical research · 2025Review
- Transport of sphingolipids by yeast Npc2 supports phase separation of the vacuole membrane.bioRxiv : the preprint server for biology · 2025Article
- Article
- Induced Pluripotent Stem Cells for the Treatment of Lysosomal Storage Disorders.Journal of inherited metabolic disease · 2025Review
- Targeting Glucosylceramide Synthase: Innovative Drug Repurposing Strategies for Lysosomal Diseases.International journal of molecular sciences · 2025Article
- Current opinion on pluripotent stem cell technology in Gaucher's disease: challenges and future prospects.Cytotechnology · 2025Review
- Key Mechanisms in Lysosome Stability, Degradation and Repair.Molecular and cellular biology · 2025Review
- Sphingolipid Analysis in Clinical Research.Methods in molecular biology (Clifton, N.J.) · 2025Article
- Advancing CNS Therapeutics: Enhancing Neurological Disorders with Nanoparticle-Based Gene and Enzyme Replacement Therapies.International journal of nanomedicine · 2025Review
- Structure Revision of Halisphingosine A via Total Synthesis and Bioactivity Studies.Angewandte Chemie (International ed. in English) · 2024Article
- UPLC-MS/MS High-Risk Screening for Sphingolipidoses Using Dried Urine Spots.Biomolecules · 2024Article
Corrections and comments
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Authors and funding
4 authors.
Funding
Abstract
Johann Ludwig Wilhelm Thudicum described sphingolipids (SLs) in the late nineteenth century, but it was only in the past fifty years that SL research surged in importance and applicability. Currently, sphingolipids and their metabolism are hotly debated topics in various biochemical fields. Similar to other macromolecular reactions, SL metabolism has important implications in health and disease in most cells. A plethora of SL-related genetic ailments has been described. Defects in SL catabolism can cause the accumulation of SLs, leading to many types of lysosomal storage diseases (LSDs) collectively called sphingolipidoses. These diseases mainly impact the neuronal and immune systems, but other systems can be affected as well. This review aims to present a comprehensive, up-to-date picture of the rapidly growing field of sphingolipid LSDs, their etiology, pathology, and potential therapeutic strategies. We first describe LSDs biochemically and briefly discuss their catabolism, followed by general aspects of the major diseases such as Gaucher, Krabbe, Fabry, and Farber among others. We conclude with an overview of the available and potential future therapies for many of the diseases. We strive to present the most important and recent findings from basic research and clinical applications, and to provide a valuable source for understanding these disorders.
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