bHEXA,bHEXS hydrolyze GM2A:SM2

Stable Identifier
R-HSA-9840884
Type
Reaction [transition]
Species
Homo sapiens
Compartment
ReviewStatus
5/5
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The beta-hexosaminidase isoforms A (bHEXA) and S (bHEXS) contain an alpha subunit (HEXA) that cleaves negatively charged substrates. Each of bHEXA and bHEXS hydrolyze the charged sulfated glycosphingolipid SM2 to form SM3. Under lysosomal conditions the reaction takes place only in the presence of both BMP (lysobisphosphatidate) and GM2AP (Hepbildikler et al., 2002).
There are three forms of hexosaminidase complexes: hexosaminidase A, B and S. The A form is a tetramer of the subunits alpha (HEXA, beta A) and beta (HEXB, beta B) dimers. The B form is a tetramer of two beta subunit dimers, and the S form a tetramer of two HEXA dimers.
Literature References
PubMed ID Title Journal Year
11707436 Physiological substrates for human lysosomal beta -hexosaminidase S

Kolzer, M, Hepbildikler, ST, Sandhoff, R, Sandhoff, K, Proia, RL

J Biol Chem 2002
Participants
Participates
Catalyst Activity

beta-N-acetylhexosaminidase activity of bHEXA,bHEXS [lysosomal lumen]

This event is regulated
Orthologous Events
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