HEXA - Beta-hexosaminidase subunit alpha - human protein (Function)
 
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HEXA »  Beta-hexosaminidase subunit alpha   [ EC 3.2.1.52 ]
 
Protein also known as:  N-acetyl-beta-glucosaminidase subunit alpha.
Gene name:  HEXA
Family name: Glycosyl hydrolase 20
Entry whose protein(s) existence is based on evidence at protein level
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GENE REF ISO

Function

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Overview 
Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. The form B is active against certain oligosaccharides. The form S has no measurable activity.  
  • CuratedUniProtKB
GO molecular function 
Beta-N-acetylhexosaminidase activitydefinition[GO:0004563]  
  • IEAEC 2 GO
Protein heterodimerization activitydefinition[GO:0046982]  
1
  • IDAMGI
GO biological process 
Adult walking behaviordefinition[GO:0007628] silver  
  • IEAOrtholog Compara
Cell deathdefinition[GO:0008219]  
  • IEAUniProtKB KW
Cell morphogenesis involved in neuron differentiationdefinition[GO:0048667] silver  
  • IEAOrtholog Compara
Ganglioside catabolic processdefinition[GO:0006689] silver  
  • IEAOrtholog Compara
Lipid storagedefinition[GO:0019915] silver  
  • IEAOrtholog Compara
Lysosome organizationdefinition[GO:0007040] silver  
  • IEAOrtholog Compara
Myelinationdefinition[GO:0042552] silver  
  • IEAOrtholog Compara
Neuromuscular process controlling balancedefinition[GO:0050885] silver  
  • IEAOrtholog Compara
Neuromuscular process controlling posturedefinition[GO:0050884] silver  
  • IEAOrtholog Compara
Sensory perception of sounddefinition[GO:0007605] silver  
  • IEAOrtholog Compara
Sexual reproductiondefinition[GO:0019953] silver  
  • IEAOrtholog Compara
Skeletal system developmentdefinition[GO:0001501] silver  
  • IEAOrtholog Compara
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC 3.2.1.52: Hydrolysis of terminal non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides.  
  • CuratedUniProtKB
According to CAZy, this protein is belongs to the following pathway:
Glycoside Hydrolase Family 20  GH20  
Pathways 
According to KEGG, this protein belongs to the following pathways:
Amino sugar and nucleotide sugar metabolism  hsa00520+3073  
Glycosaminoglycan degradation  hsa00531+3073  
Glycosphingolipid biosynthesis - ganglio series  hsa00604+3073  
Glycosphingolipid biosynthesis - globo series  hsa00603+3073  
Lysosome  hsa04142+3073  
Metabolic pathways  hsa01100+3073  
Other glycan degradation  hsa00511+3073  
According to Reactome, this protein belongs to the following pathways:
Disease  REACT_116125  
Metabolism  REACT_111217  
 
More information is available from:
 

Keywords

Molecular function 
Glycosidase  definition   [KW-0326]
Hydrolase  definition   [KW-0378]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

Enzyme and pathway databases
SABIO-RK: P06865
Other
GeneWiki: HEXA
GenomeRNAi: 3073
PRO: PR:P06865