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Protein

Beta-hexosaminidase subunit alpha

Gene

Hexa

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues.

Catalytic activityi

Hydrolysis of terminal non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei323Proton donorBy similarity1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionGlycosidase, Hydrolase

Enzyme and pathway databases

ReactomeiR-MMU-1660662 Glycosphingolipid metabolism
R-MMU-2022857 Keratan sulfate degradation
R-MMU-2024101 CS/DS degradation
R-MMU-2160916 Hyaluronan uptake and degradation

Protein family/group databases

CAZyiGH20 Glycoside Hydrolase Family 20

Names & Taxonomyi

Protein namesi
Recommended name:
Beta-hexosaminidase subunit alpha (EC:3.2.1.52)
Alternative name(s):
Beta-N-acetylhexosaminidase subunit alpha
Short name:
Hexosaminidase subunit A
N-acetyl-beta-glucosaminidase subunit alpha
Gene namesi
Name:Hexa
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 9

Organism-specific databases

MGIiMGI:96073 Hexa

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Lysosome

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 22By similarityAdd BLAST22
PropeptideiPRO_000001199523 – 88By similarityAdd BLAST66
ChainiPRO_000001199689 – 528Beta-hexosaminidase subunit alphaAdd BLAST440

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi58 ↔ 104By similarity
Glycosylationi115N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi157N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi277 ↔ 328By similarity
Glycosylationi295N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi487N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi504 ↔ 521By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein, Zymogen

Proteomic databases

EPDiP29416
MaxQBiP29416
PaxDbiP29416
PeptideAtlasiP29416
PRIDEiP29416

PTM databases

iPTMnetiP29416
PhosphoSitePlusiP29416

Expressioni

Tissue specificityi

Ubiquitous. Most abundant in testis, adrenal, epididymis and heart. Low levels seen in the liver.1 Publication

Gene expression databases

BgeeiENSMUSG00000025232 Expressed in 292 organ(s), highest expression level in ileum
CleanExiMM_HEXA
GenevisibleiP29416 MM

Interactioni

Subunit structurei

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).By similarity

GO - Molecular functioni

Protein-protein interaction databases

BioGridi200280, 1 interactor
ComplexPortaliCPX-689 Beta-hexosaminidase A complex
CPX-691 Beta-hexosaminidase S complex
IntActiP29416, 1 interactor
STRINGi10090.ENSMUSP00000026262

Structurei

3D structure databases

ProteinModelPortaliP29416
SMRiP29416
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni422 – 423Critical for hydrolyzis GM2 gangliosidesBy similarity2

Sequence similaritiesi

Belongs to the glycosyl hydrolase 20 family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiKOG2499 Eukaryota
COG3525 LUCA
GeneTreeiENSGT00390000008107
HOGENOMiHOG000157972
HOVERGENiHBG005961
InParanoidiP29416
KOiK12373
OMAiDSSFPYE
OrthoDBiEOG091G04NA
TreeFamiTF313036

Family and domain databases

Gene3Di3.30.379.10, 1 hit
InterProiView protein in InterPro
IPR025705 Beta_hexosaminidase_sua/sub
IPR015883 Glyco_hydro_20_cat
IPR017853 Glycoside_hydrolase_SF
IPR029018 Hex-like_dom2
IPR029019 HEX_eukaryotic_N
PfamiView protein in Pfam
PF00728 Glyco_hydro_20, 1 hit
PF14845 Glycohydro_20b2, 1 hit
PIRSFiPIRSF001093 B-hxosamndse_ab_euk_, 1 hit
PRINTSiPR00738 GLHYDRLASE20
SUPFAMiSSF51445 SSF51445, 1 hit
SSF55545 SSF55545, 1 hit

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P29416-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MAGCRLWVSL LLAAALACLA TALWPWPQYI QTYHRRYTLY PNNFQFRYHV
60 70 80 90 100
SSAAQAGCVV LDEAFRRYRN LLFGSGSWPR PSFSNKQQTL GKNILVVSVV
110 120 130 140 150
TAECNEFPNL ESVENYTLTI NDDQCLLASE TVWGALRGLE TFSQLVWKSA
160 170 180 190 200
EGTFFINKTK IKDFPRFPHR GVLLDTSRHY LPLSSILDTL DVMAYNKFNV
210 220 230 240 250
FHWHLVDDSS FPYESFTFPE LTRKGSFNPV THIYTAQDVK EVIEYARLRG
260 270 280 290 300
IRVLAEFDTP GHTLSWGPGA PGLLTPCYSG SHLSGTFGPV NPSLNSTYDF
310 320 330 340 350
MSTLFLEISS VFPDFYLHLG GDEVDFTCWK SNPNIQAFMK KKGFTDFKQL
360 370 380 390 400
ESFYIQTLLD IVSDYDKGYV VWQEVFDNKV KVRPDTIIQV WREEMPVEYM
410 420 430 440 450
LEMQDITRAG FRALLSAPWY LNRVKYGPDW KDMYKVEPLA FHGTPEQKAL
460 470 480 490 500
VIGGEACMWG EYVDSTNLVP RLWPRAGAVA ERLWSSNLTT NIDFAFKRLS
510 520
HFRCELVRRG IQAQPISVGY CEQEFEQT
Length:528
Mass (Da):60,613
Last modified:July 27, 2011 - v2
Checksum:i95DEA72D767094EF
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti56A → G in CAA45615 (PubMed:1379046).Curated1
Sequence conflicti56A → G in AAC53246 (PubMed:7833835).Curated1
Sequence conflicti237Q → E in X79061 (PubMed:7896264).Curated1
Sequence conflicti237Q → E in X79062 (PubMed:7896264).Curated1
Sequence conflicti455E → Q in X79061 (PubMed:7896264).Curated1
Sequence conflicti455E → Q in X79062 (PubMed:7896264).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X64331 mRNA Translation: CAA45615.1
U05837
, U05824, U05825, U05826, U05827, U05828, U05829, U05830, U05831, U05832, U05833, U05834, U05835, U05836 Genomic DNA Translation: AAC53246.1
U07721
, U07709, U07710, U07711, U07712, U07713, U07714, U07715, U07716, U07717, U07718, U07719, U07720 Unassigned DNA Translation: AAA18777.1
U07631 mRNA Translation: AAA18775.1
X79061 Genomic DNA No translation available.
X79062 Genomic DNA No translation available.
AK075895 mRNA Translation: BAC36036.1
AK075911 mRNA Translation: BAC36049.1
AK144168 mRNA Translation: BAE25744.1
AK159814 mRNA Translation: BAE35394.1
CH466522 Genomic DNA Translation: EDL25971.1
BC010755 mRNA Translation: AAH10755.1
CCDSiCCDS23250.1
PIRiI48253
RefSeqiNP_034551.2, NM_010421.5
UniGeneiMm.2284

Genome annotation databases

EnsembliENSMUST00000026262; ENSMUSP00000026262; ENSMUSG00000025232
GeneIDi15211
KEGGimmu:15211
UCSCiuc009pxw.1 mouse

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X64331 mRNA Translation: CAA45615.1
U05837
, U05824, U05825, U05826, U05827, U05828, U05829, U05830, U05831, U05832, U05833, U05834, U05835, U05836 Genomic DNA Translation: AAC53246.1
U07721
, U07709, U07710, U07711, U07712, U07713, U07714, U07715, U07716, U07717, U07718, U07719, U07720 Unassigned DNA Translation: AAA18777.1
U07631 mRNA Translation: AAA18775.1
X79061 Genomic DNA No translation available.
X79062 Genomic DNA No translation available.
AK075895 mRNA Translation: BAC36036.1
AK075911 mRNA Translation: BAC36049.1
AK144168 mRNA Translation: BAE25744.1
AK159814 mRNA Translation: BAE35394.1
CH466522 Genomic DNA Translation: EDL25971.1
BC010755 mRNA Translation: AAH10755.1
CCDSiCCDS23250.1
PIRiI48253
RefSeqiNP_034551.2, NM_010421.5
UniGeneiMm.2284

3D structure databases

ProteinModelPortaliP29416
SMRiP29416
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi200280, 1 interactor
ComplexPortaliCPX-689 Beta-hexosaminidase A complex
CPX-691 Beta-hexosaminidase S complex
IntActiP29416, 1 interactor
STRINGi10090.ENSMUSP00000026262

Protein family/group databases

CAZyiGH20 Glycoside Hydrolase Family 20

PTM databases

iPTMnetiP29416
PhosphoSitePlusiP29416

Proteomic databases

EPDiP29416
MaxQBiP29416
PaxDbiP29416
PeptideAtlasiP29416
PRIDEiP29416

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000026262; ENSMUSP00000026262; ENSMUSG00000025232
GeneIDi15211
KEGGimmu:15211
UCSCiuc009pxw.1 mouse

Organism-specific databases

CTDi3073
MGIiMGI:96073 Hexa

Phylogenomic databases

eggNOGiKOG2499 Eukaryota
COG3525 LUCA
GeneTreeiENSGT00390000008107
HOGENOMiHOG000157972
HOVERGENiHBG005961
InParanoidiP29416
KOiK12373
OMAiDSSFPYE
OrthoDBiEOG091G04NA
TreeFamiTF313036

Enzyme and pathway databases

ReactomeiR-MMU-1660662 Glycosphingolipid metabolism
R-MMU-2022857 Keratan sulfate degradation
R-MMU-2024101 CS/DS degradation
R-MMU-2160916 Hyaluronan uptake and degradation

Miscellaneous databases

PROiPR:P29416
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000025232 Expressed in 292 organ(s), highest expression level in ileum
CleanExiMM_HEXA
GenevisibleiP29416 MM

Family and domain databases

Gene3Di3.30.379.10, 1 hit
InterProiView protein in InterPro
IPR025705 Beta_hexosaminidase_sua/sub
IPR015883 Glyco_hydro_20_cat
IPR017853 Glycoside_hydrolase_SF
IPR029018 Hex-like_dom2
IPR029019 HEX_eukaryotic_N
PfamiView protein in Pfam
PF00728 Glyco_hydro_20, 1 hit
PF14845 Glycohydro_20b2, 1 hit
PIRSFiPIRSF001093 B-hxosamndse_ab_euk_, 1 hit
PRINTSiPR00738 GLHYDRLASE20
SUPFAMiSSF51445 SSF51445, 1 hit
SSF55545 SSF55545, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiHEXA_MOUSE
AccessioniPrimary (citable) accession number: P29416
Secondary accession number(s): Q64246, Q91XG3
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 1, 1993
Last sequence update: July 27, 2011
Last modified: September 12, 2018
This is version 144 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Glycosyl hydrolases
    Classification of glycosyl hydrolase families and list of entries
  3. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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