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Cryo-EM structure of the human Exostosin-1 and Exostosin-2 heterodimer in complex with UDP-GlcNAc

Source organism: Homo sapiens
Biochemical function: N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity
Biological process: endochondral bone morphogenesis
Cellular component: catalytic complex

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EC 2.4.1.224: Glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase

Reaction catalysed:
UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan = UDP + N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan
Systematic name:
UDP-N-acetyl-D-glucosamine:beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase
Alternative Name(s):
  • Alpha-N-acetylglucosaminyltransferase II
  • Glucuronyl-N-acetylglucosaminylproteoglycan alpha-1,4-N-acetylglucosaminyltransferase

EC 2.4.1.225: N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase

Reaction catalysed:
UDP-alpha-D-glucuronate + N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan = UDP + beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan
Systematic name:
UDP-alpha-D-glucuronate:N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan 4-beta-glucuronosyltransferase
Alternative Name(s):
  • Heparan glucuronyltransferase II
  • N-acetylglucosaminylproteoglycan beta-1,4-glucuronyltransferase

GO terms

Biochemical function:
Biological process:
Cellular component:

Sequence families

Pfam Protein families (Pfam)
PF09258
Domain description: Glycosyl transferase family 64 domain
Occurring in:
  1. Exostosin-1
  2. Exostosin-2
The deposited structure of PDB entry 7uqx contains 1 copy of Pfam domain PF09258 (Glycosyl transferase family 64 domain) in Exostosin-1. Showing 1 copy in chain A.
The deposited structure of PDB entry 7uqx contains 1 copy of Pfam domain PF09258 (Glycosyl transferase family 64 domain) in Exostosin-2. Showing 1 copy in chain B.

PF03016
Domain description: Exostosin family
Occurring in:
  1. Exostosin-1
  2. Exostosin-2
The deposited structure of PDB entry 7uqx contains 1 copy of Pfam domain PF03016 (Exostosin family) in Exostosin-1. Showing 1 copy in chain A.
The deposited structure of PDB entry 7uqx contains 1 copy of Pfam domain PF03016 (Exostosin family) in Exostosin-2. Showing 1 copy in chain B.

InterPro InterPro annotations
IPR029044
Domain description: Nucleotide-diphospho-sugar transferases
Occurring in:
  1. Exostosin-1
  2. Exostosin-2
IPR015338
Domain description: Glycosyl transferase 64 domain
Occurring in:
  1. Exostosin-1
  2. Exostosin-2
IPR004263
Domain description: Exostosin-like
Occurring in:
  1. Exostosin-1
  2. Exostosin-2
IPR040911
Domain description: Exostosin, GT47 domain
Occurring in:
  1. Exostosin-1
  2. Exostosin-2

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