Names & Taxonomy
- Uniprot ID:
- Q9UBQ6
- Entry Name:
- EXTL2_HUMAN
- Status:
- reviewed
- Protein Names:
- Exostosin-like 2 (EC 2.4.1.223) (Alpha-1,4-N-acetylhexosaminyltransferase EXTL2) (Alpha-GalNAcT EXTL2) (EXT-related protein 2) (Glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase) [Cleaved into: Processed exostosin-like 2]
- Gene Names:
- EXTL2 EXTR2
- Gene Names Primary:
- EXTL2
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 330
- Sequence:
- MRCCHICKLPGRVMGIRVLRLSLVVILVLLLVAGALTALLPSVKEDKMLMLRREIKSQGKSTMDSFTLIMQTYNRTDLLLKLLNHYQAVPNLHKVIVVWNNIGEKAPDELWNSLGPHPIPVIFKQQTANRMRNRLQVFPELETNAVLMVDDDTLISTPDLVFAFSVWQQFPDQIVGFVPRKHVSTSSGIYSYGSFEMQAPGSGNGDQYSMVLIGASFFNSKYLELFQRQPAAVHALIDDTQNCDDIAMNFIIAKHIGKTSGIFVKPVNMDNLEKETNSGYSGMWHRAEHALQRSYCINKLVNIYDSMPLRYSNIMISQFGFPYANYKRKI
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Endoplasmic reticulum membrane
Function
- Function:
- Glycosyltransferase required for the biosynthesis of heparan-sulfate and responsible for the alternating addition of beta-1-4-linked glucuronic acid (GlcA) and alpha-1-4-linked N-acetylglucosamine (GlcNAc) units to nascent heparan sulfate chains.
- Pathway:
- Glycan metabolism; heparan sulfate biosynthesis.
- Catalytic Activity:
- UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1->3)-beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl-proteoglycan = UDP + alpha-N-acetyl-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-(1->3)-beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl-proteoglycan.
- Cofactor:
- COFACTOR: Name=Mn(2+); Xref=ChEBI:CHEBI:29035;
- Active Site:
- ACT_SITE 245 245
- Gene Ontology Go:
- endoplasmic reticulum
endoplasmic reticulum membrane
extracellular exosome
integral component of membrane
alpha-1,4-N-acetylgalactosaminyltransferase activity
glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity
metal ion binding
glycosaminoglycan biosynthetic process
heparan sulfate proteoglycan biosynthetic process
N-acetylglucosamine metabolic process
UDP-N-acetylgalactosamine metabolic process - Gene Ontology Biological Process:
- glycosaminoglycan biosynthetic process
heparan sulfate proteoglycan biosynthetic process
N-acetylglucosamine metabolic process
UDP-N-acetylgalactosamine metabolic process - Gene Ontology Molecular Function:
- alpha-1,4-N-acetylgalactosaminyltransferase activity
glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity
metal ion binding - Gene Ontology Cellular Component:
- endoplasmic reticulum
endoplasmic reticulum membrane
extracellular exosome
integral component of membrane - Keywords:
- Complete proteome
Direct protein sequencing
Disulfide bond
Endoplasmic reticulum
Glycoprotein
Glycosyltransferase
Manganese
Membrane
Metal-binding
Reference proteome
Secreted
Signal-anchor
Transferase
Transmembrane
Transmembrane helix