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Protein

Beta-hexosaminidase subunit alpha

Gene

HEXA

Organism
Bos taurus (Bovine)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-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)LengthDescriptionGraphical viewFeature identifierActions
Active sitei323 – 3231Proton donorBy similarity

GO - Molecular functioni

  1. beta-N-acetylhexosaminidase activity Source: UniProtKB-EC

GO - Biological processi

  1. adult walking behavior Source: Ensembl
  2. carbohydrate metabolic process Source: InterPro
  3. cell morphogenesis involved in neuron differentiation Source: Ensembl
  4. ganglioside catabolic process Source: Ensembl
  5. glycosaminoglycan metabolic process Source: Ensembl
  6. lipid storage Source: Ensembl
  7. lysosome organization Source: Ensembl
  8. myelination Source: Ensembl
  9. neuromuscular process controlling balance Source: Ensembl
  10. neuromuscular process controlling posture Source: Ensembl
  11. sensory perception of sound Source: Ensembl
  12. sexual reproduction Source: Ensembl
  13. skeletal system development Source: Ensembl
Complete GO annotation...

Keywords - Molecular functioni

Glycosidase, Hydrolase

Enzyme and pathway databases

ReactomeiREACT_204575. Glycosphingolipid metabolism.
REACT_217191. CS/DS degradation.
REACT_217291. Keratan sulfate degradation.
REACT_224746. 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
ProteomesiUP000009136: Chromosome 10

Subcellular locationi

Lysosome By similarity

GO - Cellular componenti

  1. lysosome Source: UniProtKB-SubCell
  2. membrane Source: Ensembl
Complete GO annotation...

Keywords - Cellular componenti

Lysosome

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 2222By similarityAdd
BLAST
Propeptidei23 – 8866By similarityPRO_0000274203Add
BLAST
Chaini89 – 529441Beta-hexosaminidase subunit alphaPRO_0000274204Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Disulfide bondi58 ↔ 104By similarity
Glycosylationi115 – 1151N-linked (GlcNAc...)Sequence Analysis
Glycosylationi157 – 1571N-linked (GlcNAc...)Sequence Analysis
Disulfide bondi277 ↔ 328By similarity
Glycosylationi295 – 2951N-linked (GlcNAc...)Sequence Analysis
Disulfide bondi505 ↔ 522By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein, Zymogen

Proteomic databases

PRIDEiQ0V8R6.

Interactioni

Protein-protein interaction databases

STRINGi9913.ENSBTAP00000017261.

Structurei

3D structure databases

ProteinModelPortaliQ0V8R6.
SMRiQ0V8R6. Positions 23-528.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni423 – 4242Critical for hydrolyzis GM2 gangliosidesBy similarity

Sequence similaritiesi

Belongs to the glycosyl hydrolase 20 family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiCOG3525.
GeneTreeiENSGT00390000008107.
HOGENOMiHOG000157972.
HOVERGENiHBG005961.
InParanoidiQ0V8R6.
KOiK12373.
OMAiITYGPDW.
OrthoDBiEOG7KDFB6.
TreeFamiTF313036.

Family and domain databases

Gene3Di3.20.20.80. 1 hit.
3.30.379.10. 1 hit.
InterProiIPR025705. Beta_hexosaminidase_sua/sub.
IPR029018. Chitobiase/Hex_dom_2-like.
IPR015883. Glyco_hydro_20_cat-core.
IPR013781. Glyco_hydro_catalytic_dom.
IPR017853. Glycoside_hydrolase_SF.
IPR029019. HEX_eukaryotic_N.
[Graphical view]
PfamiPF00728. Glyco_hydro_20. 1 hit.
PF14845. Glycohydro_20b2. 1 hit.
[Graphical view]
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.

Cross-referencesi

Sequence databases

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

3D structure databases

ProteinModelPortaliQ0V8R6.
SMRiQ0V8R6. Positions 23-528.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi9913.ENSBTAP00000017261.

Chemistry

ChEMBLiCHEMBL1075052.

Protein family/group databases

CAZyiGH20. Glycoside Hydrolase Family 20.

Proteomic databases

PRIDEiQ0V8R6.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSBTAT00000017261; ENSBTAP00000017261; ENSBTAG00000012981.
GeneIDi504468.
KEGGibta:504468.

Organism-specific databases

CTDi3073.

Phylogenomic databases

eggNOGiCOG3525.
GeneTreeiENSGT00390000008107.
HOGENOMiHOG000157972.
HOVERGENiHBG005961.
InParanoidiQ0V8R6.
KOiK12373.
OMAiITYGPDW.
OrthoDBiEOG7KDFB6.
TreeFamiTF313036.

Enzyme and pathway databases

ReactomeiREACT_204575. Glycosphingolipid metabolism.
REACT_217191. CS/DS degradation.
REACT_217291. Keratan sulfate degradation.
REACT_224746. Hyaluronan uptake and degradation.

Miscellaneous databases

NextBioi20866675.

Family and domain databases

Gene3Di3.20.20.80. 1 hit.
3.30.379.10. 1 hit.
InterProiIPR025705. Beta_hexosaminidase_sua/sub.
IPR029018. Chitobiase/Hex_dom_2-like.
IPR015883. Glyco_hydro_20_cat-core.
IPR013781. Glyco_hydro_catalytic_dom.
IPR017853. Glycoside_hydrolase_SF.
IPR029019. HEX_eukaryotic_N.
[Graphical view]
PfamiPF00728. Glyco_hydro_20. 1 hit.
PF14845. Glycohydro_20b2. 1 hit.
[Graphical view]
PIRSFiPIRSF001093. B-hxosamndse_ab_euk_. 1 hit.
PRINTSiPR00738. GLHYDRLASE20.
SUPFAMiSSF51445. SSF51445. 1 hit.
SSF55545. SSF55545. 1 hit.
ProtoNetiSearch...

Publicationsi

  1. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].

Entry informationi

Entry nameiHEXA_BOVIN
AccessioniPrimary (citable) accession number: Q0V8R6
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 6, 2007
Last sequence update: September 5, 2006
Last modified: January 7, 2015
This is version 71 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Glycosyl hydrolases
    Classification of glycosyl hydrolase families and list of entries
  2. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.