Beta-hexosaminidase subunit alpha (Hexa) Recombinant Protein | Hexa recombinant protein

Recombinant Mouse Beta-hexosaminidase subunit alpha (Hexa)

Cat. Num. AAA963660

• Gene Names: Hexa; Hex-1
• Purity: Greater or equal to 85% purity as determined by SDS-PAGE.
• Synonyms: Beta-hexosaminidase subunit alpha (Hexa); Recombinant Mouse Beta-hexosaminidase subunit alpha (Hexa); Hexa recombinant protein

• Host: E Coli or Yeast or Baculovirus or Mammalian Cell
• Purity/Purification: Greater or equal to 85% purity as determined by SDS-PAGE.
• Form/Format: Lyophilized or liquid (Format to be determined during the manufacturing process)
• Sequence Positions: 89-528, Full length protein
• Sequence: TLGKNILVVSVVTAECNEFPNLESVENYTLTINDDQCLLASETVWGALRGLETFSQLVWKSAEGTFFINKTKIKDFPRFPHRGVLLDTSRHYLPLSSILDTLDVMAYNKFNVFHWHLVDDSSFPYESFTFPELTRKGSFNPVTHIYTAQDVKEVIEYARLRGIRVLAEFDTPGHTLSWGPGAPGLLTPCYSGSHLSGTFGPVNPSLNSTYDFMSTLFLEISSVFPDFYLHLGGDEVDFTCWKSNPNIQAFMKKKGFTDFKQLESFYIQTLLDIVSDYDKGYVVWQEVFDNKVKVRPDTIIQVWREEMPVEYMLEMQDITRAGFRALLSAPWYLNRVKYGPDWKDMYKVEPLAFHGTPEQKALVIGGEACMWGEYVDSTNLVPRLWPRAGAVAERLWSSNLTTNIDFAFKRLSHFRCELVRRGIQAQPISVGYCEQEFEQT
• Sequence Length: 440

• Species: Mouse
• Preparation and Storage: Store at -20 degrees C. For long-term storage, store at -20 degrees C or -80 degrees C. Store working aliquots at 4 degrees C for up to one week. Repeated freezing and thawing is not recommended.

Related Product Information for Hexa recombinant protein

This gene encodes the alpha 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. Alpha subunit gene mutations lead to Tay-Sachs disease (GM2-gangliosidosis type I).

NCBI and Uniprot Product Information

• NCBI GI #: 67514549
• NCBI GeneID: 15211
• NCBI Accession #: NP_034551.2
• NCBI GenBank Nucleotide #: NM_010421.5
• UniProt Accession #: P29416; Q64246; Q91XG3
• Molecular Weight: 60,613 Da
• NCBI Official Full Name: beta-hexosaminidase subunit alpha preproprotein
• NCBI Official Synonym Full Names: hexosaminidase A
• NCBI Official Symbol: Hexa
• NCBI Official Synonym Symbols: Hex-1
• NCBI Protein Information: beta-hexosaminidase subunit alpha
• UniProt Protein Name: Beta-hexosaminidase subunit alpha
• Protein Family: Beta-hexosaminidase
• UniProt Gene Name: Hexa
• UniProt Synonym Gene Names: Hexosaminidase subunit A

NCBI Description

This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mice lacking the encoded protein exhibit accumulation of gangliosides in the brain and membranous cytoplasmic bodies in neurons. Certain mutations in the human ortholog of this gene cause Tay-Sachs disease. [provided by RefSeq, Aug 2016]

Uniprot Description

Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues.