Gene/Proteome Database (LMPD)
LMPD ID
LMP000141
Gene ID
Species
Mus musculus (Mouse)
Gene Name
hexosaminidase A
Gene Symbol
Synonyms
Hex-1
Alternate Names
beta-hexosaminidase subunit alpha; hexosaminidase subunit A; beta-N-acetylhexosaminidase subunit alpha; N-acetyl-beta-glucosaminidase subunit alpha
Chromosome
9
Map Location
9 B|9 32.02 cM
EC Number
3.2.1.52
Proteins
| beta-hexosaminidase subunit alpha precursor | |
|---|---|
| Refseq ID | NP_034551 |
| Protein GI | 67514549 |
| UniProt ID | P29416 |
| mRNA ID | NM_010421 |
| Length | 528 |
| RefSeq Status | PROVISIONAL |
| MAGCRLWVSLLLAAALACLATALWPWPQYIQTYHRRYTLYPNNFQFRYHVSSAAQAGCVVLDEAFRRYRNLLFGSGSWPRPSFSNKQQTLGKNILVVSVVTAECNEFPNLESVENYTLTINDDQCLLASETVWGALRGLETFSQLVWKSAEGTFFINKTKIKDFPRFPHRGVLLDTSRHYLPLSSILDTLDVMAYNKFNVFHWHLVDDSSFPYESFTFPELTRKGSFNPVTHIYTAQDVKEVIEYARLRGIRVLAEFDTPGHTLSWGPGAPGLLTPCYSGSHLSGTFGPVNPSLNSTYDFMSTLFLEISSVFPDFYLHLGGDEVDFTCWKSNPNIQAFMKKKGFTDFKQLESFYIQTLLDIVSDYDKGYVVWQEVFDNKVKVRPDTIIQVWREEMPVEYMLEMQDITRAGFRALLSAPWYLNRVKYGPDWKDMYKVEPLAFHGTPEQKALVIGGEACMWGEYVDSTNLVPRLWPRAGAVAERLWSSNLTTNIDFAFKRLSHFRCELVRRGIQAQPISVGYCEQEFEQT | |
| sig_peptide: 1..22 inference: COORDINATES: ab initio prediction:SignalP:4.0 calculated_mol_wt: 2219 peptide sequence: MAGCRLWVSLLLAAALACLATA mat_peptide: 89..528 product: Beta-hexosaminidase subunit alpha experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (P29416.2) calculated_mol_wt: 50429 peptide sequence: TLGKNILVVSVVTAECNEFPNLESVENYTLTINDDQCLLASETVWGALRGLETFSQLVWKSAEGTFFINKTKIKDFPRFPHRGVLLDTSRHYLPLSSILDTLDVMAYNKFNVFHWHLVDDSSFPYESFTFPELTRKGSFNPVTHIYTAQDVKEVIEYARLRGIRVLAEFDTPGHTLSWGPGAPGLLTPCYSGSHLSGTFGPVNPSLNSTYDFMSTLFLEISSVFPDFYLHLGGDEVDFTCWKSNPNIQAFMKKKGFTDFKQLESFYIQTLLDIVSDYDKGYVVWQEVFDNKVKVRPDTIIQVWREEMPVEYMLEMQDITRAGFRALLSAPWYLNRVKYGPDWKDMYKVEPLAFHGTPEQKALVIGGEACMWGEYVDSTNLVPRLWPRAGAVAERLWSSNLTTNIDFAFKRLSHFRCELVRRGIQAQPISVGYCEQEFEQT | |
Gene Information
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005764 | IDA:MGI | C | lysosome |
| GO:0016020 | IDA:MGI | C | membrane |
| GO:0004563 | IDA:MGI | F | beta-N-acetylhexosaminidase activity |
| GO:0060395 | IDA:MGI | P | SMAD protein signal transduction |
| GO:0007628 | IMP:MGI | P | adult walking behavior |
| GO:0005975 | IEA:InterPro | P | carbohydrate metabolic process |
| GO:0048667 | IMP:MGI | P | cell morphogenesis involved in neuron differentiation |
| GO:0006689 | IMP:MGI | P | ganglioside catabolic process |
| GO:0030203 | IGI:MGI | P | glycosaminoglycan metabolic process |
| GO:0019915 | IMP:MGI | P | lipid storage |
| GO:0007626 | IGI:MGI | P | locomotory behavior |
| GO:0007040 | IMP:MGI | P | lysosome organization |
| GO:0042552 | IGI:MGI | P | myelination |
| GO:0050885 | IGI:MGI | P | neuromuscular process controlling balance |
| GO:0050884 | IMP:MGI | P | neuromuscular process controlling posture |
| GO:0007605 | IGI:MGI | P | sensory perception of sound |
| GO:0019953 | IMP:MGI | P | sexual reproduction |
| GO:0001501 | IGI:MGI | P | skeletal system development |
KEGG Pathway Links
Domain Information
InterPro Annotations
| Accession | Description |
|---|---|
| IPR029019 | Beta-hexosaminidase, eukaryotic type, N-terminal |
| IPR025705 | Beta_hexosaminidase_sua/sub |
| IPR029018 | Chitobiase/beta-hexosaminidase domain 2-like |
| IPR015883 | Glycoside hydrolase family 20, catalytic core |
| IPR017853 | Glycoside hydrolase superfamily |
| IPR013781 | Glycoside hydrolase, catalytic domain |
UniProt Annotations
Entry Information
Comments
| Comment Type | Description |
|---|---|
| Catalytic Activity | Hydrolysis of terminal non-reducing N-acetyl- D-hexosamine residues in N-acetyl-beta-D-hexosaminides. |
| Function | Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. |
| Similarity | Belongs to the glycosyl hydrolase 20 family. {ECO:0000305}. |
| Subcellular Location | Lysosome. |
| Subunit | There are 3 forms of beta-hexosaminidase: hexosaminidase A is a trimer composed of one alpha chain, one beta-A chain and one beta-B chain; hexosaminidase B is a tetramer of two beta-A and two beta-B chains; hexosaminidase S is a homodimer of two alpha chains. The two beta chains are derived from the cleavage of a precursor chain (By similarity). {ECO:0000250}. |
| Tissue Specificity | Ubiquitous. Most abundant in testis, adrenal, epididymis and heart. Low levels seen in the liver. {ECO:0000269|PubMed:7896264}. |
Identical and Related Proteins
Unique RefSeq proteins for LMP000141 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 67514549 | RefSeq | NP_034551 | 528 | beta-hexosaminidase subunit alpha precursor |
Identical Sequences to LMP000141 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:67514549 | DBBJ | BAC36036.1 | 528 | unnamed protein product [Mus musculus] |
| GI:67514549 | DBBJ | BAC36049.1 | 528 | unnamed protein product [Mus musculus] |
| GI:67514549 | DBBJ | BAE25744.1 | 528 | unnamed protein product [Mus musculus] |
| GI:67514549 | DBBJ | BAE35394.1 | 528 | unnamed protein product [Mus musculus] |
| GI:67514549 | GenBank | EDL25971.1 | 528 | hexosaminidase A [Mus musculus] |
| GI:67514549 | SwissProt | P29416.2 | 528 | RecName: Full=Beta-hexosaminidase subunit alpha; AltName: Full=Beta-N-acetylhexosaminidase subunit alpha; Short=Hexosaminidase subunit A; AltName: Full=N-acetyl-beta-glucosaminidase subunit alpha; Flags: Precursor [Mus musculus] |
Related Sequences to LMP000141 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:67514549 | DBBJ | BAE35457.1 | 528 | unnamed protein product [Mus musculus] |
| GI:67514549 | DBBJ | BAE30831.1 | 528 | unnamed protein product [Mus musculus] |
| GI:67514549 | DBBJ | BAE35636.1 | 528 | unnamed protein product [Mus musculus] |
| GI:67514549 | DBBJ | BAE29566.1 | 528 | unnamed protein product [Mus musculus] |
| GI:67514549 | EMBL | CAA45615.1 | 528 | beta-N-acetylhexosaminidase [Mus musculus] |
| GI:67514549 | GenBank | AAC53246.1 | 528 | beta-N-acetylhexosaminidase, alpha-subunit [Mus musculus] |