Hmdb loader
Identification
HMDB Protein ID HMDBP13568
Secondary Accession Numbers None
Name Beta-hexosaminidase subunit alpha
Synonyms
  1. Beta-N-acetylhexosaminidase subunit alpha
  2. N-acetyl-beta-glucosaminidase subunit alpha
  3. Hexosaminidase subunit A
Gene Name HEXA
Protein Type Unknown
Biological Properties
General Function Not Available
Specific Function 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.
Pathways
  • Amino sugar and nucleotide sugar metabolism
  • Glycosaminoglycan degradation
  • Glycosphingolipid biosynthesis - ganglio series
  • Glycosphingolipid biosynthesis - globo and isoglobo series
  • Lysosome
  • Other glycan degradation
  • Various types of N-glycan biosynthesis
Reactions Not Available
GO Classification
Biological Process
myelination
sensory perception of sound
locomotory behavior
neuromuscular process controlling balance
glycosaminoglycan biosynthetic process
lipid storage
glycosaminoglycan metabolic process
SMAD protein signal transduction
adult walking behavior
neuromuscular process controlling posture
skeletal system development
cell morphogenesis involved in neuron differentiation
ganglioside catabolic process
sexual reproduction
carbohydrate metabolic process
lysosome organization
Cellular Component
cytosol
azurophil granule
lysosome
membrane
intracellular membrane-bounded organelle
Molecular Function
N-acetyl-beta-D-galactosaminidase activity
beta-N-acetylhexosaminidase activity
acetylglucosaminyltransferase activity
protein heterodimerization activity
Cellular Location Not Available
Gene Properties
Chromosome Location Not Available
Locus Not Available
SNPs Not Available
Gene Sequence Not Available
Protein Properties
Number of Residues 528
Molecular Weight 60612.605
Theoretical pI 6.536
Pfam Domain Function
Signals
  • 1-22;
Transmembrane Regions Not Available
Protein Sequence Not Available
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID P29416
UniProtKB/Swiss-Prot Entry Name HEXA_MOUSE
PDB IDs Not Available
GenBank Gene ID Not Available
GeneCard ID Not Available
GenAtlas ID Not Available
HGNC ID Not Available
References
General References
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  4. Beccari T, Hoade J, Orlacchio A, Stirling JL: Cloning and sequence analysis of a cDNA encoding the alpha-subunit of mouse beta-N-acetylhexosaminidase and comparison with the human enzyme. Biochem J. 1992 Jul 15;285 ( Pt 2):593-6. doi: 10.1042/bj2850593. [PubMed:1379046 ]
  5. Bianconi S, Beccari T, Stirling JL, Sheardown S, Orlacchio A: Organization of the gene for the alpha-subunit of mouse beta-N-acetylhexosaminidase (HEXa). Biochem Mol Biol Int. 1994 Oct;34(3):579-86. [PubMed:7833835 ]
  6. Yamanaka S, Johnson ON, Norflus F, Boles DJ, Proia RL: Structure and expression of the mouse beta-hexosaminidase genes, Hexa and Hexb. Genomics. 1994 Jun;21(3):588-96. doi: 10.1006/geno.1994.1318. [PubMed:7959736 ]
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