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TGF-beta receptor type-2; transforming growth factor-beta receptor type II; TGF-beta receptor type II; TGF-beta type II receptor; TbetaR-II; TGFR-2 (TGFBR2)
Function:
- receptor for TGF-beta
- TGF-beta receptor-1 & TGF-beta receptor-2 form heteromeric complex after binding of TGF-beta
- on ligand binding, forms a receptor complex consisting of two type II & two type I transmembrane S/T kinases
- the type II receptors phosphorylate & activate type I receptors which autophosphorylate, then bind & activate SMAD transcriptional regulators
- binds to DAXX
- interacts with TCTEX1D4
- associates with betaglycan
- phosphorylated on a Ser/Thr residue in the cytoplasmic domain
- TGF-beta receptor-1 & TGF-beta receptor-2 form heteromeric complex after binding of TGF-beta
Cofactor: Mg+2 or Mn+2 (putative)
Structure:
- belongs to the protein kinase superfamily, TKL Ser/Thr protein kinase family, TGFB receptor subfamily
- contains 1 protein kinase domain
Compartment: membrane
Alternative splicing: named isoforms=2
Pathology:
1) defects in TGFBR2 are the cause of cancer syndromes
a) hereditary nonpolyposis colorectal cancer type 6
b) esophageal cancer
2) defects in TGFBR1 are the cause of syndromes associated with aortic aneurysm
a) Loeys-Dietz syndrome type 1B
b) Loeys-Dietz syndrome type 2B
c) aortic aneurysm familial thoracic type 3
Interactions
molecular events
General
glycoprotein
phosphoprotein
protein subunit
serine/threonine kinase receptor
tgf beta receptor
Properties
SIZE: entity length = 567 aa
MW = 65 kD
COMPARTMENT: cellular membrane
STATE: active state
MOTIF: signal sequence {1-22}
cysteine residue {C51}
MODIFICATION: cysteine residue {C84}
cysteine residue {C54}
MODIFICATION: cysteine residue {C71}
cysteine residue {C61}
MODIFICATION: cysteine residue {C67}
cysteine residue {C67}
MODIFICATION: cysteine residue {C61}
N-glycosylation site {N70}
cysteine residue {C71}
MODIFICATION: cysteine residue {C54}
cysteine residue {C77}
MODIFICATION: cysteine residue {C101}
cysteine residue {C84}
MODIFICATION: cysteine residue {C51}
N-glycosylation site {N94}
cysteine residue {C101}
MODIFICATION: cysteine residue {C77}
cysteine residue {C121}
MODIFICATION: cysteine residue {C136}
cysteine residue {C136}
MODIFICATION: cysteine residue {C121}
cysteine residue {C138}
MODIFICATION: cysteine residue {C143}
cysteine residue {C143}
MODIFICATION: cysteine residue {C138}
N-glycosylation site {N154}
transmembrane domain {167-187}
kinase domain
SITE: 244-544
MOTIF: ATP-binding site
NAME: ATP-binding site
SITE: 250-258
ATP-binding site
NAME: ATP-binding site
SITE: 277-277
aspartate residue {D379}
Ser phosphorylation site {S548}
Database Correlations
OMIM correlations
MORBIDMAP 190182
UniProt P37173
PFAM correlations
Entrez Gene 7048
Kegg hsa:7048
ENZYME 2.7.11.30
References
- UniProt :accession P37173
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=TGFBR2
Component-of
transforming Growth Factor [TGF]-beta receptor complex