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Protein

Beta-hexosaminidase subunit beta

Gene

HEXB

Organism
Felis catus (Cat) (Felis silvestris catus)
Status
Reviewed-Annotation score: Annotation score: 5 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.

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 sitei329 – 3291Proton donorBy similarity

GO - Molecular functioni

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

GO - Biological processi

  1. astrocyte cell migration Source: Ensembl
  2. cellular calcium ion homeostasis Source: Ensembl
  3. cellular protein metabolic process Source: Ensembl
  4. ganglioside catabolic process Source: Ensembl
  5. glycosaminoglycan metabolic process Source: Ensembl
  6. lipid storage Source: Ensembl
  7. locomotory behavior Source: Ensembl
  8. lysosome organization Source: Ensembl
  9. male courtship behavior Source: Ensembl
  10. myelination Source: Ensembl
  11. neuromuscular process controlling balance Source: Ensembl
  12. oligosaccharide catabolic process Source: Ensembl
  13. oogenesis Source: Ensembl
  14. penetration of zona pellucida Source: Ensembl
  15. phospholipid biosynthetic process Source: Ensembl
  16. positive regulation of transcription from RNA polymerase II promoter Source: Ensembl
  17. regulation of cell shape Source: Ensembl
  18. sensory perception of sound Source: Ensembl
  19. skeletal system development Source: Ensembl
Complete GO annotation...

Keywords - Molecular functioni

Glycosidase, Hydrolase

Enzyme and pathway databases

BRENDAi3.2.1.52. 2235.

Protein family/group databases

CAZyiGH20. Glycoside Hydrolase Family 20.

Names & Taxonomyi

Protein namesi
Recommended name:
Beta-hexosaminidase subunit beta (EC:3.2.1.52)
Alternative name(s):
Beta-N-acetylhexosaminidase subunit beta
Short name:
Hexosaminidase subunit B
N-acetyl-beta-glucosaminidase subunit beta
Gene namesi
Name:HEXB
OrganismiFelis catus (Cat) (Felis silvestris catus)
Taxonomic identifieri9685 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaLaurasiatheriaCarnivoraFeliformiaFelidaeFelinaeFelis
ProteomesiUP000011712 Componenti: Unplaced

Subcellular locationi

GO - Cellular componenti

  1. acrosomal vesicle Source: Ensembl
  2. extracellular vesicular exosome Source: Ensembl
  3. lysosome Source: UniProtKB-SubCell
  4. membrane Source: Ensembl
Complete GO annotation...

Keywords - Cellular componenti

Lysosome

Pathology & Biotechi

Involvement in diseasei

Defects in HEXB are responsible for Sandhoff disease (GM2-gangliosidosis type-II). This disorder is found in Korat cats (initially imported from Thailand).

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 2424Sequence AnalysisAdd
BLAST
Chaini25 – 531507Beta-hexosaminidase subunit betaPRO_0000012001Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Glycosylationi50 – 501N-linked (GlcNAc...)Sequence Analysis
Disulfide bondi65 ↔ 111By similarity
Glycosylationi116 – 1161N-linked (GlcNAc...)Sequence Analysis
Glycosylationi164 – 1641N-linked (GlcNAc...)Sequence Analysis
Disulfide bondi283 ↔ 334By similarity
Glycosylationi301 – 3011N-linked (GlcNAc...)Sequence Analysis
Disulfide bondi508 ↔ 525By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein

Structurei

3D structure databases

ProteinModelPortaliP49614.
SMRiP49614. Positions 29-526.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the glycosyl hydrolase 20 family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiCOG3525.
HOVERGENiHBG005961.
InParanoidiP49614.
KOiK12373.

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.

P49614-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MRHRGLGLAA LLALLAAVAP RSSAAAGAAL WPMPLSVKTS PRLLHLSRDN
60 70 80 90 100
FSIGYGPSST AGPTCSLLQE AFRRYHEYIF GFDKRQRRPA KPNSAIELQQ
110 120 130 140 150
LLVTVVLDSE CDLFPNITSD ESYTLLVKEP VAFLKANRVW GVLRGLETFS
160 170 180 190 200
QLIYQDSYGT FTVNESDIID SPRFPHRGIL IDTARHFLPV KSILKTLDAM
210 220 230 240 250
AFNKFNVLHW HIVDDQSFPY QSVTFPELSN KGSYSLSHVY TPNDVHTVIE
260 270 280 290 300
YARLRGIRVI PEFDSPGHTQ SWGKGQKDLL TPCYNEHKQS GTFGPINPIL
310 320 330 340 350
NSTYNFLSQF FKEVSMVFPD HFVHLGGDEV EFQCWESNPE IQGFMKQKGF
360 370 380 390 400
GKDFRRLESF YLQKLLGIVS TVKKGSIVWQ EVFDDHVKLL PGTIVQVWKN
410 420 430 440 450
QVYTEELREV TAAGFPVILS APWYLDWISY GQDWRNYYKV DPLHFDGSQE
460 470 480 490 500
QKKLVIGGEA CLWGEFVDAT NLTPRLWPRA SAVGERLWSP EDITSVGNAY
510 520 530
NRLTVHRCRM VRRGISAEPL FTGYCDYEYK T
Length:531
Mass (Da):60,427
Last modified:May 10, 2005 - v2
Checksum:iAC6E0C8C6509509B
GO

Sequence cautioni

The sequence AAB30707.2 differs from that shown. Reason: Frameshift at position 27. Curated

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
S70340 mRNA. Translation: AAB30707.2. Frameshift.
RefSeqiNP_001009333.2. NM_001009333.2.

Genome annotation databases

GeneIDi493928.
KEGGifca:493928.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
S70340 mRNA. Translation: AAB30707.2. Frameshift.
RefSeqiNP_001009333.2. NM_001009333.2.

3D structure databases

ProteinModelPortaliP49614.
SMRiP49614. Positions 29-526.
ModBaseiSearch...
MobiDBiSearch...

Protein family/group databases

CAZyiGH20. Glycoside Hydrolase Family 20.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

GeneIDi493928.
KEGGifca:493928.

Organism-specific databases

CTDi3074.

Phylogenomic databases

eggNOGiCOG3525.
HOVERGENiHBG005961.
InParanoidiP49614.
KOiK12373.

Enzyme and pathway databases

BRENDAi3.2.1.52. 2235.

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. "Characterization of the molecular defect in a feline model for type II GM2-gangliosidosis (Sandhoff disease)."
    Muldoon L.L., Neuwelt E.A., Pagel M.A., Weiss D.L.
    Am. J. Pathol. 144:1109-1118(1994) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Strain: Korat.
    Tissue: Liver.

Entry informationi

Entry nameiHEXB_FELCA
AccessioniPrimary (citable) accession number: P49614
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 1, 1996
Last sequence update: May 10, 2005
Last modified: April 1, 2015
This is version 90 of the entry and version 2 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.