Beta-hexosaminidase subunit alpha

Definition:

Hydrolyzes the non-reducing end N-acetyl-D-hexosamine and/or sulfated N-acetyl-D-hexosamine of glycoconjugates, such as the oligosaccharide moieties from proteins and neutral glycolipids, or from certain mucopolysaccharides. The isozyme S is as active as the isozyme A on the anionic bis-sulfated glycans, the chondroitin-6-sulfate trisaccharide (C6S-3), and the dermatan sulfate pentasaccharide, and the sulfated glycosphingolipid SM2. The isozyme B does not hydrolyze each of these substrates, however hydrolyzes efficiently neutral oligosaccharide. Only the isozyme A is responsible for the degradation of GM2 gangliosides in the presence of GM2A.

References:

[1]. Stefan T Hepbildikler, et al. Physiological substrates for human lysosomal beta -hexosaminidase S. J Biol Chem. 2002 Jan 25;277(4):2562-72. [Content Brief]

[2]. A Novak, et al. Classification of disorders of GM2 ganglioside hydrolysis using 3H-GM2 as substrate. Biochim Biophys Acta. 1994 Mar 2;1199(2):215-23. [Content Brief]

[3]. Y Hou, et al. Direct determination of the substrate specificity of the alpha-active site in heterodimeric beta-hexosaminidase A. Biochemistry. 1996 Apr 2;35(13):3963-9. [Content Brief]

[4]. Y Hou, et al. A Pro504 --> Ser substitution in the beta-subunit of beta-hexosaminidase A inhibits alpha-subunit hydrolysis of GM2 ganglioside, resulting in chronic Sandhoff disease. J Biol Chem. 1998 Aug 14;273(33):21386-92. [Content Brief]

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