EXTL3 Glycosyltransferase which regulates the biosynthesis of heparan sulfate (HS). Important for both skeletal development and hematopoiesis, through the formation of HS proteoglycans (HSPGs). Required for the function of REG3A in regulating keratinocyte proliferation and differentiation. Belongs to the glycosyltransferase 47 family. Ubiquitous. Expressed in keratinocytes. Note: This description may include information from UniProtKB.
Protein type: EC; Glycan Metabolism - heparan sulfate biosynthesis; Membrane protein, integral; Transferase
Chromosomal Location of human Ortholog: 8p21.1
Cellular Component:  endoplasmic reticulum; endoplasmic reticulum membrane; Golgi apparatus; integral component of membrane
Molecular Function:  glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity; metal ion binding; transferase activity, transferring glycosyl groups
Biological Process:  heparan sulfate proteoglycan biosynthetic process; IRE1-mediated unfolded protein response; positive regulation of cell growth; protein glycosylation
Disease: Immunoskeletal Dysplasia With Neurodevelopmental Abnormalities
Reference #:  O43909 (UniProtKB)
Alt. Names/Synonyms: BOTV; DKFZp686C2342; epididymis secretory sperm binding protein; exostoses (multiple)-like 3; exostosin like glycosyltransferase 3; exostosin tumor-like 3; Exostosin-like 3; EXT-related 1; EXT-related protein 1; EXTL1L; EXTL3; EXTR1; Glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Hereditary multiple exostoses gene isolog; ISDNA; KIAA0519; Multiple exostosis-like protein 3; Putative tumor suppressor protein EXTL3; REG; reg receptor; REGR; RPR
Gene Symbols: EXTL3
Molecular weight: 104,749 Da
Basal Isoelectric point: 6.08  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  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  InnateDB