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

Entrez Gene ID
Gene Name
hexosaminidase A
Gene Symbol
Species
Mus musculus

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

KEGG Pathway ID Description
mmu00520 Amino sugar and nucleotide sugar metabolism
mmu00603 Glycosphingolipid biosynthesis - globo series
mmu04142 Lysosome

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

Gene Name
hexosaminidase A
Protein Entry
HEXA_MOUSE
UniProt ID
Species
Mouse

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]