Sphingolipid Degradation (Lysosomal)
- Compartment
- Lysosome
- Main tissue
- All cells; neurons, macrophages
- Rate-limiting
- Not flagged in the source
- Steps
- 6
Reaction steps
In source order, 6 total
-
1
Sphingomyelin + H2O -> ceramide + phosphocholine
› Notes
Deficiency causes Niemann-Pick disease types A/B (cherry-red macula, hepatosplenomegaly, foam cells).
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2
GM2 ganglioside -> GM3 ganglioside + N-acetylgalactosamine
› Notes
HEXA deficiency causes Tay-Sachs disease (cherry-red macula, no hepatosplenomegaly); HEXA+HEXB deficiency causes Sandhoff disease.
-
3
Globotriaosylceramide (Gb3) -> lactosylceramide + galactose
› Notes
X-linked deficiency causes Fabry disease (angiokeratomas, neuropathic pain, renal failure).
-
4
Glucosylceramide -> ceramide + glucose
› Notes
Deficiency causes Gaucher disease, the commonest lysosomal storage disorder (Gaucher cells, bone crises, pancytopenia).
-
5
Galactocerebroside -> ceramide + galactose
› Notes
Deficiency causes Krabbe disease (globoid cell leukodystrophy).
-
6
Sulfatide -> galactocerebroside + sulfate
In Sulfatide› Notes
Deficiency causes metachromatic leukodystrophy.
Showing all 6 steps.
Recent literature
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Europe PMC · fetched just now · sorted by publication date
-
1
Countering postoperative immune suppression with a self-assembling dendritic cell nanovaccine.
Yang Q, Wang T, Zhang H, Xu Y, Huang S, He Y, Wei R, Yin N, Gao Y, Yang L, Jin S, Mi L, Chao R, Li M, Feng M,… · 2026-05-09
open access unreviewed -
2
Next-Generation Sequencing and Variant Cataloguing for Screening and Diagnosis of Mucolipidoses and Other Lysosome-Related Organelle Disorders, Including Lysosomal Membrane or Transport Disorders
Vlasova-St. Louis I, Khaiboullina S. · 2026-05-31
open access unreviewed -
3
Gut-Derived Lipid Mediators Orchestrate Ovarian Metabolic Homeostasis and Clutch Persistence in Aging Laying Hens via the PLA2G6-ALOX15B-AGPAT3 Axis.
Li X, Wang X, Cai X, Meng Q, Sun Y, Yang C, Yao J. · 2026-05-11
open access unreviewed -
4
Transport of sphingolipids by yeast Npc2 supports phase separation of the vacuole membrane.
Kim H, Lipp NF, Juarez-Contreras I, Wong AM, Budin I. · 2026-03-16
open access unreviewed -
5
Chalcone and <i>Trans</i>-Chalcone Induce Transcriptomic Changes in <i>Caenorhabditis elegans</i> Compatible with a Novel Cumulative Damage Mode of Action.
Galli G, Bruun CS, García-Estrada C, Balaña-Fouce R, Martinez-Valladares M, Hansen TVA. · 2026-04-24
open access unreviewed -
6
SPNS2 exports sphingosine-1-phosphate and imports glucose.
Weigel C, Hossen ML, Brown RDR, Senkal CE, Green CD, Newton J, Saha S, Palladino END, Ni B, Celi FS, Fang X, … · 2026-04-20
cited 2× open access unreviewed -
7
Dapagliflozin induces renal lipidomic remodeling and systemic metabolic improvement.
Li A, Ye Y, Tang J, Hu J, Shi M, Wu Y, Ji C, Zhang H. · 2026-04-17
open access unreviewed -
8
Elevated serum glucosylsphingosine level in children with obesity: relation to plasma atherogenesis.
Salah NY, Abdel Hakam D, Abdullah FA, Hamza MS, Ramadan EA, Mahmoud R. · 2026-01-22
open access unreviewed -
9
AXL Promotes Ischemic Myelin Repair Through Alleviating Myelin Debris Deposition and Lipid Droplets Accumulation.
Jia J, Gan Y, Li J, Li L, Meng H, Sun M, Ye L, Mao R, Cao X, Xia S, Bao X, Liu R, Zhang M, Xu Y. · 2026-01-12
cited 1× open access unreviewed -
10
Establishing an untargeted lipidomics workflow for cellular analysis: insights into endothelial cell function in anaphylaxis.
Delgado Dolset MI, Escolar-Peña A, Fernández-Bravo S, García-Cívico AJ, Pajares L, Neuhaus R, González-Mendio… · 2026-03-04
open access unreviewed
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.