HEXB Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. Belongs to the glycosyl hydrolase 20 family. Note: This description may include information from UniProtKB.
Protein type: Carbohydrate Metabolism - amino sugar and nucleotide sugar; EC; Glycan Metabolism - glycosaminoglycan degradation; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Glycan Metabolism - other glycan degradation; Hydrolase
Chromosomal Location of human Ortholog: 5q13.3
Cellular Component:  acrosomal vesicle; azurophil granule; azurophil granule lumen; extracellular region; lysosomal lumen
Molecular Function:  acetylglucosaminyltransferase activity; beta-N-acetylhexosaminidase activity; N-acetyl-beta-D-galactosaminidase activity; protein binding; protein heterodimerization activity; protein homodimerization activity
Biological Process:  astrocyte cell migration; cellular calcium ion homeostasis; cellular protein metabolic process; chondroitin sulfate catabolic process; ganglioside catabolic process; glycosphingolipid metabolic process; hyaluronan catabolic process; keratan sulfate catabolic process; lipid storage; locomotory behavior; lysosome organization; male courtship behavior; myelination; neuromuscular process controlling balance; neutrophil degranulation; oligosaccharide catabolic process; oogenesis; penetration of zona pellucida; phospholipid biosynthetic process; positive regulation of transcription by RNA polymerase II; regulation of cell shape; sensory perception of sound; skeletal system development
Disease: Sandhoff Disease
Reference #:  P07686 (UniProtKB)
Alt. Names/Synonyms: beta-hexosaminidase beta-subunit; Beta-hexosaminidase subunit beta; Beta-hexosaminidase subunit beta chain A; Beta-hexosaminidase subunit beta chain B; Beta-N-acetylhexosaminidase subunit beta; Cervical cancer proto-oncogene 7 protein; ENC-1AS; epididymis luminal protein 248; epididymis secretory protein Li 111; epididymis secretory sperm binding protein; HCC-7; HEL-248; HEL-S-111; HEXB; hexosaminidase B (beta polypeptide); Hexosaminidase subunit B; hexosaminidase subunit beta; N-acetyl-beta-glucosaminidase subunit beta
Gene Symbols: HEXB
Molecular weight: 63,137 Da
Basal Isoelectric point: 6.29  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  AlphaFold  |  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  ENZYME  |  Phospho3D  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene