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MS
unreviewed

beta-Hexosaminidase A (HEXA) + GM2 activator

1 reactions · 1 pathways

Clinical / pharmacological. Tay-Sachs disease

What it does, reaction by reaction

1 reactions

Sphingolipid Degradation (Lysosomal) Lipid Metabolism · Lysosome

step 2 Irreversible/directional

GM2 ganglioside -> GM3 ganglioside + N-acetylgalactosamine

Converts GM2 ganglioside into GM3 ganglioside N-acetylgalactosamine

Notes

HEXA deficiency causes Tay-Sachs disease (cherry-red macula, no hepatosplenomegaly); HEXA+HEXB deficiency causes Sandhoff disease.

Showing all 1 reactions.

What accelerates and inhibits it

Regulation is pathway-specific, so each context is listed separately

0 entries

No regulation recorded for this enzyme in the source documents.

Recent literature

Europe PMC · from cache · sorted by publication date

  1. 1
    Structure-function analysis of empasiprubart, a calcium- and pH-dependent clinical phase complement C2 blocking antibody.

    Bracke L, Gytz Olesen H, Pannecoucke E, Delahaye T, Persson EK, Blanchetot C, de Haard H, Hack CE, Andersen G… · 2026-05-04

    open access unreviewed
  2. 2
    Post-licensure safety of nirsevimab from the Canadian National Vaccine Safety (CANVAS) network.

    Viñeta Paramo M, Kiely M, Valiquette L, Muller MP, McGeer A, Isenor JE, Sadarangani M, Kellner JD, Vanderkooi… · 2026-04-28

    open access unreviewed
  3. 3
    Long-term immune protection after infant hepatitis B vaccination in children.

    Tarigan Girsang R, Fadlyana E, Dwi Putra MG, Rusmil K, Setiabudiawan B, Kartasamita CB, Febfi T. · 2026-04-16

    open access unreviewed
  4. 4
    Costs of managing adverse reactions to hexavalent vaccines: Comparison of DT3aP-HBV-IPV/Hib and DT2aP-HBV-IPV-Hib in France and Spain.

    Nikitas G, Mohy A, Duchenne M, Genovez V, Casabona G, Hennessy E, Vallejo L, Haddouche AA, Marijam A. · 2026-03-16

    open access unreviewed
  5. 5
    Beyond a single species: Mapping virulence traits across the redefined <i>Fusobacterium nucleatum</i> complex.

    Wolf M, Scholz KJ, Al-Ahmad A, Steinberg T, Kruse A, Rezasoltani S, Cieplik F, Conrads G. · 2026-02-24

    cited 2× open access unreviewed
  6. 6
    A scoping review of turmeric adulteration based on data from six continents.

    Gafner S, Orhan N, Kahraman Ç, Blumenthal M. · 2025-12-26

    open access unreviewed
  7. 7
    Engineered protein nanoclusters reduce liver fibrosis and hepatocellular carcinoma in mice models.

    Saxena T, Guedes G, Domingues I, Yagoubi H, Guilbaud L, Van den Bossche W, Pangua C, Vande Velde G, Ucakar B,… · 2026-05-27

    open access unreviewed
  8. 8
  9. 9
    Novel 1H-pyrrole-2,5-dione derivatives as potential anti-diabetic agents: design, synthesis, in vitro, in ovo, and molecular docking studies.

    Gezer D, Ozyıldız Z, Gokmen Z, Dagsuyu E, Sacan O, Akar RO, Yıldırım EG, Ulukaya E, Adacan K, Kutlu A. · 2026-06-13

    unreviewed
  10. 10
    MicroLC-HRMS selective quantification of a biotherapeutic fusion protein in plasma and cerebrospinal fluid of patients with Lafora disease using a linker-containing signature peptide.

    Esposito E, Caravelli A, Muccioli L, Cancellerini C, Tappatà M, Pizzi E, Minardi R, DEFEAT-LD Study Group, Ca… · 2026-05-19

    unreviewed

External claims. These come from an index outside this database and are not checked against it. Treat them as leads.