UniProt:P07686 HEXB

chain
  • signal peptide:1-42
  • propeptide:43-121
  • chain:122-556
  • chain:122-311
  • chain:315-556
checksum B3A0A36594F62536
comment
  • 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 (PubMed:11707436, PubMed:9694901, PubMed:8672428, PubMed:8123671). The isozyme B does not hydrolyze each of these substrates, however hydrolyzes efficiently neutral oligosaccharide (PubMed:11707436). Only the isozyme A is responsible for the degradation of GM2 gangliosides in the presence of GM2A (PubMed:9694901, PubMed:8672428, PubMed:8123671). During fertilization is responsible, at least in part, for the zona block to polyspermy. Present in the cortical granules of non-activated oocytes, is exocytosed during the cortical reaction in response to oocyte activation and inactivates the sperm galactosyltransferase-binding site, accounting for the block in sperm binding to the zona pellucida (By similarity).ACTIVITY REGULATION Addition of GM2A stimulates the hydrolysis of sulfated glycosphingolipid SM2 and the ganglioside GM2.SUBUNIT There are 3 forms of beta-hexosaminidase: hexosaminidase A is an heterodimer composed of one subunit alpha and one subunit beta (chain A and B); hexosaminidase B is an homodimer of two beta subunits (two chains A and B); hexosaminidase S is a homodimer of two alpha subunits (By similarity). The composition of the dimer (isozyme A versus isozyme S) has a significant effect on the substrate specificity of the alpha subunit active site (PubMed:8672428).PTM N-linked glycans at Asn-142 and Asn-190 consist of Man(3)-GlcNAc(2) and Man(5 to 7)-GlcNAc(2), respectively.PTM The beta-A and beta-B chains are produced by proteolytic processing of the precursor beta chain.SIMILARITY Belongs to the glycosyl hydrolase 20 family.
crossReference
databaseName UniProt
dbId 56474
description
  • recommendedName: fullName evidence="23"Beta-hexosaminidase subunit beta ecNumber evidence="6 16 18 21"3.2.1.52/ecNumber alternativeName: Beta-N-acetylhexosaminidase subunit beta shortName:Hexosaminidase subunit B alternativeName: Cervical cancer proto-oncogene 7 protein shortName:HCC-7 alternativeName: N-acetyl-beta-glucosaminidase subunit beta component recommendedName: Beta-hexosaminidase subunit beta chain B /component component recommendedName: Beta-hexosaminidase subunit beta chain A /component
displayName UniProt:P07686 HEXB
geneName
  • HEXB
  • HCC7
identifier P07686
isSequenceChanged false
keyword
  • 3D-structure
  • Cytoplasmic vesicle
  • Direct protein sequencing
  • Disease variant
  • Disulfide bond
  • Gangliosidosis
  • Glycoprotein
  • Glycosidase
  • Hydrolase
  • Lysosome
  • Neurodegeneration
  • Reference proteome
  • Signal
  • Zymogen
modified [InstanceEdit:11703169] Weiser, JD, 2021-09-15
name
  • HEXB
otherIdentifier
  • 11715837_a_at
  • 16986203
  • 201944_at
  • 2815804
  • 2815805
  • 2815806
  • 2815807
  • 2815808
  • 2815810
  • 2815815
  • 2815816
  • 2815817
  • 2815820
  • 2815822
  • 2815824
  • 2815825
  • 2815826
  • 2815830
  • 2815832
  • 2815833
  • 2815834
  • 2815837
  • 2815839
  • 2815841
  • 2815842
  • 2815843
  • 2815844
  • 3074
  • 8106252
  • A_14_P100715
  • A_14_P119296
  • GE60505
  • GO:0000003
  • GO:0000902
  • GO:0001501
  • GO:0001669
  • GO:0002376
  • GO:0003674
  • GO:0004553
  • GO:0004563
  • GO:0005515
  • GO:0005575
  • GO:0005576
  • GO:0005615
  • GO:0005622
  • GO:0005737
  • GO:0005764
  • GO:0005773
  • GO:0005975
  • GO:0006629
  • GO:0006687
  • GO:0006689
  • GO:0006790
  • GO:0006810
  • GO:0006874
  • GO:0007040
  • GO:0007338
  • GO:0007341
  • GO:0007605
  • GO:0007626
  • GO:0008049
  • GO:0008150
  • GO:0008152
  • GO:0008360
  • GO:0008375
  • GO:0008654
  • GO:0009056
  • GO:0009058
  • GO:0009313
  • GO:0016020
  • GO:0016192
  • GO:0016757
  • GO:0016787
  • GO:0016798
  • GO:0019915
  • GO:0019953
  • GO:0030133
  • GO:0030154
  • GO:0030203
  • GO:0030207
  • GO:0030214
  • GO:0031323
  • GO:0031410
  • GO:0034641
  • GO:0035578
  • GO:0040011
  • GO:0042340
  • GO:0042552
  • GO:0042582
  • GO:0042592
  • GO:0042802
  • GO:0043202
  • GO:0043226
  • GO:0043312
  • GO:0043615
  • GO:0044267
  • GO:0044281
  • GO:0045944
  • GO:0048477
  • GO:0048856
  • GO:0048870
  • GO:0050877
  • GO:0050885
  • GO:0050905
  • GO:0060473
  • GO:0070062
  • GO:0102148
  • HMNXSV003058045
  • M23294_at
  • PH_hs_0022175
  • TC05000359.hg
  • TC05002451.hg
  • g13128866_3p_s_at
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • HEXB_HUMAN
sequenceLength 556
species [Species:48887] Homo sapiens
url http://purl.uniprot.org/uniprot/P07686

Referrals

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(referenceSequence)
(interactor)
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