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Protein

Beta-hexosaminidase subunit alpha

Gene

HEXA

Organism
Bos taurus (Bovine)
Status
Reviewed-Annotation score: -Experimental evidence at transcript 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.By similarity

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

BRENDAi3.2.1.52 908
ReactomeiR-BTA-1660662 Glycosphingolipid metabolism
R-BTA-2022857 Keratan sulfate degradation
R-BTA-2024101 CS/DS degradation
R-BTA-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
OrganismiBos taurus (Bovine)
Taxonomic identifieri9913 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaLaurasiatheriaCetartiodactylaRuminantiaPecoraBovidaeBovinaeBos
Proteomesi
  • UP000009136 Componenti: Chromosome 10

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

Pathology & Biotechi

Chemistry databases

ChEMBLiCHEMBL1075052

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 22By similarityAdd BLAST22
PropeptideiPRO_000027420323 – 88By similarityAdd BLAST66
ChainiPRO_000027420489 – 529Beta-hexosaminidase subunit alphaAdd BLAST441

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
Disulfide bondi505 ↔ 522By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein, Zymogen

Proteomic databases

PaxDbiQ0V8R6
PRIDEiQ0V8R6

Expressioni

Gene expression databases

BgeeiENSBTAG00000012981

Interactioni

Protein-protein interaction databases

STRINGi9913.ENSBTAP00000017261

Structurei

3D structure databases

ProteinModelPortaliQ0V8R6
SMRiQ0V8R6
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni423 – 424Critical 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
InParanoidiQ0V8R6
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.

Q0V8R6-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAGSTLRFSL LLAAAFAGRA TALWPWPQYI QTSELRYTIF PQSFQFQYHL
60 70 80 90 100
SSAAQVGCSV LDEAFQRYRD LLFGSVAFRF PHPIEKRHTS EKNSLVVLVV
110 120 130 140 150
TPGCDQFPSL GSVENYTLTI NDEQSLLLSE TVWGALRGLE TFSQLIWRSP
160 170 180 190 200
EGTFYVNKTD IEDFPRFPHR GLLLDTSRHY LPLASILDTL DVMAYNKFNV
210 220 230 240 250
FHWHLVDDSS FPYESFTFPE LTKKGSYNPA THIYTAQDVK EVIEYARLRG
260 270 280 290 300
IRVLAEFDTP GHTLSWGPGV PGLLTPCYSG SHPSGTFGPV NPALNNTYEF
310 320 330 340 350
MSTFFLEIST VFPDFYLHLG GDEVDFTCWK SNPDIQAFMK KKGFGDDFKK
360 370 380 390 400
LESFYIQTLL DIVSAYGKGY VVWQEVFDNK VKVRPDTIIQ VWREEIPVKY
410 420 430 440 450
VKELALVTRA GFRALLSAPW YLNHITYGPD WKEIYLVEPL AFEGSPEQKA
460 470 480 490 500
LVIGGEACMW GEYVDSTNLV PRLWPRAGAV AERLWSNKMV SNLDFAFKRL
510 520
AHFRCELLRR GVQAQPLSVG YCDMEFEQT
Length:529
Mass (Da):60,353
Last modified:September 5, 2006 - v1
Checksum:i878641B502FCD3A9
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
BT026152 mRNA Translation: ABG66991.1
RefSeqiNP_001068632.1, NM_001075164.2
UniGeneiBt.6065

Genome annotation databases

EnsembliENSBTAT00000017261; ENSBTAP00000017261; ENSBTAG00000012981
GeneIDi504468
KEGGibta:504468

Similar proteinsi

Entry informationi

Entry nameiHEXA_BOVIN
AccessioniPrimary (citable) accession number: Q0V8R6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 6, 2007
Last sequence update: September 5, 2006
Last modified: March 28, 2018
This is version 92 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

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