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Protein

Beta-hexosaminidase subunit alpha

Gene

HEXA

Organism
Pongo abelii (Sumatran orangutan) (Pongo pygmaeus abelii)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Protein inferred from homologyi

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
  14. SMAD protein signal transduction Source: Ensembl
Complete GO annotation...

Keywords - Molecular functioni

Glycosidase, Hydrolase

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
OrganismiPongo abelii (Sumatran orangutan) (Pongo pygmaeus abelii)
Taxonomic identifieri9601 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaePongo
ProteomesiUP000001595 Componenti: Chromosome 15

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 – 2222Sequence AnalysisAdd
BLAST
Propeptidei23 – 8866By similarityPRO_0000011997Add
BLAST
Chaini89 – 529441Beta-hexosaminidase subunit alphaPRO_0000011998Add
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

PRIDEiQ5RC84.

Structurei

3D structure databases

ProteinModelPortaliQ5RC84.
SMRiQ5RC84. 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

GeneTreeiENSGT00390000008107.
HOVERGENiHBG005961.
InParanoidiQ5RC84.
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.

Q5RC84-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MASSRLWFSL LLAAALAGRA TALWPWPQNI QTSDQRYVLY PNNFQFQYDV
60 70 80 90 100
SSAAQPGCSV LDEAFQRYRD LLFGSGSWPR PYLTGKRHTL EKNVLVVSVV
110 120 130 140 150
TPGCNQLPTL ESVENYTLTI NDDQCLLLSE TVWGALRGLE TFSQLVWKSA
160 170 180 190 200
EGTFFINKTE IEDFPRFPHR GLLLDTSRHY LPLSSILDTL DVMAYNKLNV
210 220 230 240 250
FHWHLVDDPS FPYESFTFPE LMRKGSYNPV THIYTAQDVK EVIEYARLRG
260 270 280 290 300
IRVLAEFDTP GHTLSWGPGI PGLLTPCYSG SEPSGTFGPV NPSLNNTYEF
310 320 330 340 350
MSTFFLEVSS VFPDFYLHLG GDEVDFTCWK SNPDIQDFMR KKGFGEDFKQ
360 370 380 390 400
LESFYIQTLL DIVSSYGKGY VVWQEVFDNK VKIRPDTIIQ VWREDIPVNY
410 420 430 440 450
MKELELVTKA GFRALLSAPW YLNRISYGPD WKDFYVVEPL AFEGTPEQKA
460 470 480 490 500
LVIGGEACMW GEYVDNTNLV PRLWPRAGAV AERLWSNKLT SDLTFAYERL
510 520
SHFRCELLRR GVQAQPLNVG FCEQEFEQT
Length:529
Mass (Da):60,605
Last modified:December 20, 2004 - v1
Checksum:i8D00068F3EBDBA74
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
CR858396 Transcribed RNA. Translation: CAH90623.1.
RefSeqiNP_001192280.1. NM_001205351.1.

Genome annotation databases

EnsembliENSPPYT00000007808; ENSPPYP00000007503; ENSPPYG00000006616.
GeneIDi100438794.
KEGGipon:100438794.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
CR858396 Transcribed RNA. Translation: CAH90623.1.
RefSeqiNP_001192280.1. NM_001205351.1.

3D structure databases

ProteinModelPortaliQ5RC84.
SMRiQ5RC84. Positions 23-528.
ModBaseiSearch...
MobiDBiSearch...

Protein family/group databases

CAZyiGH20. Glycoside Hydrolase Family 20.

Proteomic databases

PRIDEiQ5RC84.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSPPYT00000007808; ENSPPYP00000007503; ENSPPYG00000006616.
GeneIDi100438794.
KEGGipon:100438794.

Organism-specific databases

CTDi3073.

Phylogenomic databases

GeneTreeiENSGT00390000008107.
HOVERGENiHBG005961.
InParanoidiQ5RC84.
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.
ProtoNetiSearch...

Publicationsi

  1. The German cDNA consortium
    Submitted (OCT-2004) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Brain cortex.

Entry informationi

Entry nameiHEXA_PONAB
AccessioniPrimary (citable) accession number: Q5RC84
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 9, 2005
Last sequence update: December 20, 2004
Last modified: March 3, 2015
This is version 69 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.