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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 · fetched just now · sorted by publication date

  1. 1
    Seroprevalence of hepatitis E in blood donors in Santiago, Chile: a monocentric cross-sectional study.

    Porte L, Weitzel IB, Martínez-Valdebenito C, Pizarro G, Bustamante V, Weitzel T. · 2026-08-22

    open access unreviewed
  2. 2
    Structural and functional characterisation of the dextran utilisome from <i>Bacteroides thetaiotaomicron</i>.

    Feasey M, Silale A, Baslé A, van den Berg B. · 2026-07-17

    cited 2× open access unreviewed
  3. 3
    Exploring toothpaste ingredients as potential chemical kindling agents in neuropsychiatric disorders: An adult zebrafish (<i>Danio rerio</i>) study.

    Mathew T, Bhardwaj S, Goyal A, Bhatt A, Mahadevan A, Kashif MS, Karau PB, Mitra D, V V B, Pullikan J, Jose J,… · 2026-07-10

    open access unreviewed
  4. 4
    Temperature-assisted stabilization of aqueous polychlorinated biphenyl stock solutions for sorption experiments.

    Števuľová R, Vadlamudi SK, Štibrányi L, Híveš J. · 2026-06-09

    open access unreviewed
  5. 5
    Immunological insights into why pertussis continues to be endemic.

    Tanriover MD, Forsyth K, He Q, Diavatopoulos DA, Orozco Fernández R, Hozbor DF, Middleton DB, Muloiwa R, Muño… · 2026-06-30

    open access unreviewed
  6. 6
    Early antibiotic exposure and vaccine immune responses in preterm infants: potential sex-specific differences.

    Haag L, Dietz-Ziegler S, Schwarz J, Kaiser G, Rühle J, Hebel J, Lesk T, Lajqi T, Müller J, Schoppmeier U, Fun… · 2026-06-27

    open access unreviewed
  7. 7
    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

    cited 1× open access unreviewed
  8. 8
    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

    cited 1× open access unreviewed
  9. 9
    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
  10. 10
    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

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