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Tchem
HEXB
Beta-hexosaminidase subunit beta

Protein Classes
Protein Summary
Description
Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II). Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2014]
Uniprot Accession IDs
Gene Name
Ensembl ID
  • ENST00000261416
  • ENSP00000261416
  • ENSG00000049860

Symbol
  • ENC-1AS
  • HEL-248
  • HEL-S-111
Illumination Graph
Knowledge Table
Most Knowledge About
Knowledge Value (0 to 1 scale)
kinase perturbation
1
disease perturbation
0.99
biological process
0.97
gene perturbation
0.85
protein domain
0.85


IDG Development Level Summary
Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 120.31   (req: < 5)
Gene RIFs: 39   (req: <= 3)
Antibodies: 234   (req: <= 50)
Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 120.31   (req: >= 5)
Gene RIFs: 39   (req: > 3)
Antibodies: 234   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 28
Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligand: 1
Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 0
Active Ligands (1)
1 – 1 of 1
CHEMBL257158
chemical structure image
Protein Data Bank (9)
1 – 5 of 9
PDB Structure Id
Ligand
Method
Resolution (Å)
M.W. (kDa)
Pub Year
Title
PDB Structure Id
M.W.
Resolution
Pub Year
Pathways (28)
CS/DS degradation (R-HSA-2024101)

Click on a row in the table to change the structure displayed.

Items per page:
1 – 5 of 19
Data Source
Name
Explore in Pharos
Explore in Source
Reactome
CS/DS degradation
Reactome
Chondroitin sulfate/dermatan sulfate metabolism
Reactome
Defective HEXB causes GM2G2
Reactome
Disease
Reactome
Diseases associated with glycosaminoglycan metabolism
Name
Explore in Pharos
Explore in Source
CS/DS degradation
Chondroitin sulfate/dermatan sulfate metabolism
Defective HEXB causes GM2G2
Disease
Diseases associated with glycosaminoglycan metabolism
Gene Ontology Terms (35)
Items per page:
10
1 – 5 of 5
GO Term
Evidence
Assigned by
Inferred from Direct Assay (IDA)
UniProtKB
Inferred from Direct Assay (IDA)
MGI
Inferred from Direct Assay (IDA)
MGI
Traceable Author Statement (TAS)
Reactome
Inferred from Electronic Annotation (IEA)
UniProtKB-EC
Protein-Protein Interactions (139)
1 – 10 of 139
POTEB
Tdark
Novelty: 2.20408163
p_int: 0.999999294
p_ni: 6.64e-7
p_wrong: 4.2e-8
Data Source: BioPlex
POTEB2
Tdark
Novelty: 2.20408163
p_int: 0.999587572
p_ni: 0.000412425
p_wrong: 3e-9
Data Source: BioPlex
HEXA
Tchem
Family: Enzyme
Novelty: 0.00302415
p_int: 0.959308414
p_ni: 0.040691587
Score: 0.994
Data Source: BioPlex,Reactome,STRINGDB
APOD
Tbio
Novelty: 0.00362142
p_int: 0.895415705
p_ni: 0.104309684
p_wrong: 0.000274611
Score: 0.198
Data Source: BioPlex,STRINGDB
FUCA2
Tchem
Family: Enzyme
Novelty: 0.02818887
Score: 0.977
Data Source: STRINGDB
GM2A
Tbio
Novelty: 0.0083165
Score: 0.972
Data Source: Reactome,STRINGDB
GLB1
Tchem
Family: Enzyme
Novelty: 0.00274332
Score: 0.964
Data Source: STRINGDB
FUCA1
Tchem
Family: Enzyme
Novelty: 0.00435966
Score: 0.961
Data Source: STRINGDB
GLA
Tclin
Family: Enzyme
Novelty: 0.0006641
Score: 0.957
Data Source: STRINGDB
GUSB
Tchem
Family: Enzyme
Novelty: 0.00074055
Score: 0.947
Data Source: STRINGDB
Publication Statistics
PubMed Score  120.31

PubMed score by year
PubTator Score  149.5

PubTator score by year
Amino Acid Sequence
Residue Counts
Protein Sequence
ProtVista Viewer
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