Active arginine residues in beta-hexosaminidase. Identification through studies of the B1 variant of Tay-Sachs disease.

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Article

Active arginine residues in beta-hexosaminidase. Identification through studies of the B1 variant of Tay-Sachs disease.

The Journal of biological chemistry - 25 Aug 1991

Brown C A, Mahuran D J

Abstract excerpt

Lysosomal beta-hexosaminidase (EC 3.2.1.52) occurs as two major isoenzymes, hexosaminidases A (alpha beta) and B (beta beta). The alpha- and beta-subunits are encoded by the HEXA and HEXB genes, respectively. Extensive homology in both the gene structures and deduced primary sequences demonstrate their common evolutionary origin. Defects in the alpha- or beta-subunits lead to Tay-Sachs of Sandhoff disease,...

Topics

Arginine
Base Sequence
Blotting, Western
Catalysis
Cell Line
Diacetyl
Hexosaminidase A
Hexosaminidase B
Humans
Hydrogen-Ion Concentration
Isoenzymes
Kinetics

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Lysosomes

Molecular Sequence Data

Mutation

Phenylglyoxal

Tay-Sachs Disease

Transfection

Source

Open in PubMed

Identifiers

PMID: 1831451

Genes

HEXA
HEXB

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 29 Aug 2026. Prepared record publication-1831451-2026-08-29.1. Manifest publication-graph-cohort-2026-08-29.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 29 Aug 2026.

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Active arginine residues in beta-hexosaminidase. Identification through studies of the B1 variant of Tay-Sachs disease.PMID 1831451Read ↗

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