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).
-
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
Association Between the Root Canal Microbiome and Apical Lesion Size: An Observational Shotgun Metagenomic Study.
Pérez-Carrasco V, Uroz-Torres D, Soriano-Lerma A, Soriano M, García-Salcedo JA, Arias-Moliz MT. · 2026-06-02
cited 1× open access unreviewed -
2
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 -
3
Interplay of GBA1 with lysosomal dysfunction and inflammation in Parkinson's disease.
Wang R, Hatano T, Hattori N, Cossu D. · 2025-12-30
cited 7× open access unreviewed -
4
Species-dependent activities of the PINK1-parkin axis.
Huang W, Chen K, Wen J, Zhang T, Li S, Li XJ, Yang W. · 2026-09-18
open access unreviewed -
5
A Contemporary Pathomechanistic Nosology of Inherited Lysosomal Disorders.
McCarron EP, Blau N, Eden ER, Platt FM, Aerts JM, Jones SA, Ferreira CR, Stepien KM. · 2026-09-01
cited 1× unreviewed -
6
Disruption of sphingolipid metabolism promotes tau seeding through endolysosomal membrane rigidification and rupture.
Tittelmeier J, Sandhof CA, Martin N, El-Kabarity D, Ngonza-Nito SB, Melki R, Nussbaum-Krammer C. · 2026-08-27
cited 3× open access unreviewed -
7
CircRNA Profiling Identifies Candidates Associated with Abdominal Fat Deposition in Ducks.
Zhou T, Yao J, Mao J, Yang C, Gu S, Jiang Y, Bai H, Chang G, Chen G, Wang Z. · 2026-08-17
open access unreviewed -
8
The riddle of sphingolipid structure-function relationship in the nervous system.
Muñoz-Gil S, Spassieva SD. · 2026-06-16
open access unreviewed -
9
Aldehyde Detoxification in Cancer: Metabolic Guardians of the Genome.
James BT, Kosmeder EP, Wang M. · 2026-07-06
open access unreviewed -
10
Metabolomic Insights into Lysosomal Storage Diseases: An Untargeted View.
Di Carlo G, Tommolini ML, Frisco A, Spalluto G, Foley D, Zucchelli M, Cufaro MC, Cicalini I, Bucci I, Federic… · 2026-07-08
cited 1× open access unreviewed
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