Gene/Proteome Database (LMPD)
LMPD ID
LMP013194
Gene ID
Species
Rattus norvegicus (Rat)
Gene Name
hexosaminidase A
Gene Symbol
Alternate Names
beta-hexosaminidase subunit alpha; hexosaminidase subunit A; Hexose aminidase A (alpha polypeptide); beta-N-acetylhexosaminidase subunit alpha; N-acetyl-beta-glucosaminidase subunit alpha;
Chromosome
8
Map Location
8q24
EC Number
3.2.1.52
Summary
mouse homolog is the alpha subunit of lysosomal beta-hexosaminidase A [RGD, Feb 2006]
Orthologs
Proteins
| beta-hexosaminidase subunit alpha precursor | |
|---|---|
| Refseq ID | NP_001004443 |
| Protein GI | 52138739 |
| UniProt ID | Q641X3 |
| mRNA ID | NM_001004443 |
| Length | 528 |
| MAGCRLWVSLLLAAALACLATALWPWPQYIQTSHRRYTLYPNNFQFRYHAGSAAQAGCVVLDEAFRRYRSLLFGSGSWPRPSFSKKQQPLGKNILMVSVVTAECNEFPNLESVENYTLTINDDQCLLSSETVWGALRGLETFSQLVWKSAEGTFFINKTKITDFPRFPHRGILLDTSRHYLPLSSILNTLDVMAYNKFNVFHWHLVDDSSFPYESFTFPELTRKGSFNPVTHIYTAQDVKEVIEYARLRGIRVLAEFDTPGHTLSWGAGVPGLLTPCYSGSRLSGTYGPVNPSLNSTYDFMSTFFLEISSVFPDFYLHLGGDEVDFTCWKSNPNIQAFMKKKGFTDYKQLESFYIQTLLDIVSDYDKGYVVWQEVFDNKVKVRPDTIIQVWREEMPVQYMKEIEAITQAGFRALLSAPWYLNRVKYGPDWKEMYKVEPLAFRGTPAQKALVIGGEACMWGEYVDSTNLVPRLWPRAGAIAERLWSSNLTTNMDFAFKRLSHFRCELLRRGIQAQPISVGYCEQEFEHT | |
| 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 (Q641X3.1) calculated_mol_wt: 50501 peptide sequence: PLGKNILMVSVVTAECNEFPNLESVENYTLTINDDQCLLSSETVWGALRGLETFSQLVWKSAEGTFFINKTKITDFPRFPHRGILLDTSRHYLPLSSILNTLDVMAYNKFNVFHWHLVDDSSFPYESFTFPELTRKGSFNPVTHIYTAQDVKEVIEYARLRGIRVLAEFDTPGHTLSWGAGVPGLLTPCYSGSRLSGTYGPVNPSLNSTYDFMSTFFLEISSVFPDFYLHLGGDEVDFTCWKSNPNIQAFMKKKGFTDYKQLESFYIQTLLDIVSDYDKGYVVWQEVFDNKVKVRPDTIIQVWREEMPVQYMKEIEAITQAGFRALLSAPWYLNRVKYGPDWKEMYKVEPLAFRGTPAQKALVIGGEACMWGEYVDSTNLVPRLWPRAGAIAERLWSSNLTTNMDFAFKRLSHFRCELLRRGIQAQPISVGYCEQEFEHT | |
Gene Information
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005764 | IEA:UniProtKB-KW | C | lysosome |
| GO:0016020 | IEA:Ensembl | C | membrane |
| GO:0004563 | IEA:UniProtKB-EC | F | beta-N-acetylhexosaminidase activity |
| GO:0007628 | IEA:Ensembl | P | adult walking behavior |
| GO:0005975 | IEA:InterPro | P | carbohydrate metabolic process |
| GO:0048667 | IEA:Ensembl | P | cell morphogenesis involved in neuron differentiation |
| GO:0006689 | IEA:Ensembl | P | ganglioside catabolic process |
| GO:0030203 | IEA:Ensembl | P | glycosaminoglycan metabolic process |
| GO:0019915 | IEA:Ensembl | P | lipid storage |
| GO:0007040 | IEA:Ensembl | P | lysosome organization |
| GO:0042552 | IEA:Ensembl | P | myelination |
| GO:0050885 | IEA:Ensembl | P | neuromuscular process controlling balance |
| GO:0050884 | IEA:Ensembl | P | neuromuscular process controlling posture |
| GO:0007605 | IEA:Ensembl | P | sensory perception of sound |
| GO:0019953 | IEA:Ensembl | P | sexual reproduction |
| GO:0001501 | IEA:Ensembl | P | skeletal system development |
KEGG Pathway Links
| KEGG Pathway ID | Description |
|---|---|
| ko00520 | Amino sugar and nucleotide sugar metabolism |
| rno00520 | Amino sugar and nucleotide sugar metabolism |
| ko00531 | Glycosaminoglycan degradation |
| rno00531 | Glycosaminoglycan degradation |
| ko00604 | Glycosphingolipid biosynthesis - ganglio series |
| rno00604 | Glycosphingolipid biosynthesis - ganglio series |
| ko00603 | Glycosphingolipid biosynthesis - globo series |
| rno00603 | Glycosphingolipid biosynthesis - globo series |
| M00079 | Keratan sulfate degradation |
| ko04142 | Lysosome |
| rno04142 | Lysosome |
| rno01100 | Metabolic pathways |
| ko00511 | Other glycan degradation |
| rno00511 | Other glycan degradation |
REACTOME Pathway Links
| REACTOME Pathway ID | Description |
|---|---|
| 5954487 | CS/DS degradation |
| 5954488 | Chondroitin sulfate/dermatan sulfate metabolism |
| 5953253 | Disease |
| 5953252 | Glycogen storage diseases |
| 5953861 | Glycosaminoglycan metabolism |
| 5954485 | Glycosphingolipid metabolism |
| 5954547 | Hyaluronan metabolism |
| 5954548 | Hyaluronan uptake and degradation |
| 5954494 | Keratan sulfate degradation |
| 5954345 | Keratan sulfate/keratin metabolism |
| 5953870 | MPS I - Hurler syndrome |
| 5953869 | MPS II - Hunter syndrome |
| 5953872 | MPS IIIA - Sanfilippo syndrome A |
| 5953864 | MPS IIIB - Sanfilippo syndrome B |
| 5953867 | MPS IIIC - Sanfilippo syndrome C |
| 5953871 | MPS IIID - Sanfilippo syndrome D |
| 5953866 | MPS IV - Morquio syndrome A |
| 5953873 | MPS IV - Morquio syndrome B |
| 5953862 | MPS IX - Natowicz syndrome |
| 5953865 | MPS VI - Maroteaux-Lamy syndrome |
| 5953868 | MPS VII - Sly syndrome |
| 5953250 | Metabolism |
| 5953249 | Metabolism of carbohydrates |
| 5953289 | Metabolism of lipids and lipoproteins |
| 5953863 | Mucopolysaccharidoses |
| 5953251 | Myoclonic epilepsy of Lafora |
| 5954267 | Sphingolipid metabolism |
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 |
| Subcellular Location | Lysosome . |
Identical and Related Proteins
Unique RefSeq proteins for LMP013194 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 52138739 | RefSeq | NP_001004443 | 528 | beta-hexosaminidase subunit alpha precursor |
Identical Sequences to LMP013194 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:52138739 | GenBank | AAH82097.1 | 528 | Hexosaminidase A [Rattus norvegicus] |
| GI:52138739 | GenBank | EDL95699.1 | 528 | hexosaminidase A, isoform CRA_a [Rattus norvegicus] |
| GI:52138739 | SwissProt | Q641X3.1 | 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 [Rattus norvegicus] |
Related Sequences to LMP013194 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:52138739 | DBBJ | BAE25744.1 | 528 | unnamed protein product [Mus musculus] |
| GI:52138739 | GenBank | AAH82097.1 | 528 | Hexosaminidase A [Rattus norvegicus] |
| GI:52138739 | GenBank | EDL95699.1 | 528 | hexosaminidase A, isoform CRA_a [Rattus norvegicus] |
| GI:52138739 | SwissProt | Q641X3.1 | 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 [Rattus norvegicus] |