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Details on Person The beta-hexosaminidase isoforms A (bHEXA) and S (bHEXS) con...

Class:IdSummation:9840887
_displayNameThe beta-hexosaminidase isoforms A (bHEXA) and S (bHEXS) con...
_timestamp2023-08-27 07:21:08
created[InstanceEdit:9840892] Stephan, Ralf, 2023-08-01
modified[InstanceEdit:9842002] Stephan, Ralf, 2023-08-16
[InstanceEdit:9842402] Stephan, Ralf, 2023-08-27
textThe 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.
(summation)[Reaction:9840884] bHEXA,bHEXS hydrolyze GM2A:SM2 [Homo sapiens]
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No pathways have been reviewed or authored by The beta-hexosaminidase isoforms A (bHEXA) and S (bHEXS) con... (9840887)