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Recombinant Human HEXB

Cat.No.: HEXB-27728TH
Product Overview: Recombinant full length Human HEXB with N-terminal proprietary tag. Predicted MW 87.23kDa.
Description: Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines.Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes.Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases.Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses.Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II).
Protein length: 556 amino acids
Molecular Weight: 87.230kDa inclusive of tags
Source: Wheat germ
Form: Liquid
Purity: Proprietary Purification
Storage buffer: pH: 8.00Constituents:0.3% Glutathione, 0.79% Tris HCl
Storage: Shipped on dry ice. Upon delivery aliquot and store at -80oC. Avoid freeze / thaw cycles.
Sequences of amino acids: MELCGLGLPRPPMLLALLLATLLAAMLALLTQVALVVQVA EAARAPSVSAKPGPALWPLPLSVKMTPNLLHLAPENFY ISHSPNSTAGPSCTLLEEAFRRYHGYIFGFYKWHHEPAEF QAKTQVQQLLVSITLQSECDAFPNISSDESYTLLVKEP VAVLKANRVWGALRGLETFSQLVYQDSYGTFTINESTIID SPRFSHRGILIDTSRHYLPVKIILKTLDAMAFNKFNVL HWHIVDDQSFPYQSITFPELSNKGSYSLSHVYTPNDVRMV IEYARLRGIRVLPEFDTPGHTLSWGKGQKDLLTPCYSR QNKLDSFGPINPTLNTTYSFLTTFFKEISEVFPDQFIHLGGDEVEFKCWESNPKIQDFMRQKGFGTDFKKLESFYIQK VLDIIATINKGSIVWQEVFDDKAKLAPGTIVEVWKDSA YPEELSRVTASGFPVILSAPWYLDLISYGQDWRKYYKVEP LDFGGTQKQKQLFIGGEACLWGEYVDATNLTPRLWPRA SAVGERLWSSKDVRDMDDAYDRLTRHRCRMVERGIAAQPL YAGYCNHENM
Sequence Similarities: Belongs to the glycosyl hydrolase 20 family.
Gene Name: HEXB hexosaminidase B (beta polypeptide) [ Homo sapiens ]
Official Symbol: HEXB
Synonyms: HEXB; hexosaminidase B (beta polypeptide); beta-hexosaminidase subunit beta;
Gene ID: 3074
mRNA Refseq: NM_000521
Protein Refseq: NP_000512
MIM: 606873
Uniprot ID: P07686
Chromosome Location: 5q13.3
Pathway: Amino sugar and nucleotide sugar metabolism, organism-specific biosystem; Amino sugar and nucleotide sugar metabolism, conserved biosystem; Glycosaminoglycan degradation, organism-specific biosystem; Glycosaminoglycan degradation, conserved biosystem; Glycosphingolipid biosynthesis - ganglio series, organism-specific biosystem;
Function: beta-N-acetylglucosaminidase activity; beta-N-acetylhexosaminidase activity; catalytic activity; cation binding; protein heterodimerization activity;

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