beta-Hexosaminidase A (HEXA) + GM2 activator
Clinical / pharmacological. Tay-Sachs disease
What it does, reaction by reaction
Sphingolipid Degradation (Lysosomal) Lipid Metabolism · Lysosome
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
No regulation recorded for this enzyme in the source documents.
Recent literature
Europe PMC · fetched just now · sorted by publication date
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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
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
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
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
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
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
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
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
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
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.