TGFBR2 Chromosome 3

Transforming growth factor beta receptor 2
234 variants 234 Health Risk

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What This Gene Does
The protein encoded by this gene is a transmembrane protein that has a protein kinase domain, forms a heterodimeric complex with TGF-beta receptor type-1, and binds TGF-beta. This receptor/ligand complex phosphorylates proteins, which then enter the nucleus and regulate the transcription of genes related to cell proliferation, cell cycle arrest, wound healing, immunosuppression, and tumorigenesis. Mutations in this gene have been associated with Marfan Syndrome, Loeys-Deitz Aortic Aneurysm Syndrome, and the development of various types of tumors. Alternatively spliced transcript variants encoding different isoforms have been characterized. [provided by RefSeq, Aug 2017]
Gene Info
Gene Group
Type 2 receptor serine/threonine kinases
Locus Type
gene with protein product
Location
3p24.1
Ensembl
ENSG00000163513
Associated Conditions (27)
Diabetic retinopathy
Familial thoracic aortic aneurysm and aortic dissection
Loeys-Dietz syndrome 2
Loeys-Dietz syndrome
Colorectal cancer
hereditary nonpolyposis
type 6
Congenital aneurysm of ascending aorta
Marfan syndrome
Connective tissue disorder
Ehlers-Danlos syndrome
TGFBR2-related disorder
Malignant tumor of esophagus
Loeys-Dietz syndrome 1
Multiple congenital anomalies/dysmorphic syndrome
Ascending tubular aorta aneurysm
Vascular dilatation
Thoracic aortic aneurysm
Thoracic aortic dissection
Cardiovascular phenotype
+7 more conditions
Key Variants
RS1002352692
Conflicting classifications of pathogenicity
Diabetic retinopathy, Diabetic retinopathy
Health Risk
RS1014929115
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS1040386724
Conflicting classifications of pathogenicity
Loeys-Dietz syndrome 2, Diabetic retinopathy, Loeys-Dietz syndrome 2
Health Risk
RS104893813
Conflicting classifications of pathogenicity
Loeys-Dietz syndrome 2, Loeys-Dietz syndrome, Colorectal cancer
Health Risk
RS1057520957
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Loeys-Dietz syndrome 2, Diabetic retinopathy
Health Risk
RS1060501985
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection, Diabetic retinopathy
Health Risk
RS112215250
Conflicting classifications of pathogenicity
Congenital aneurysm of ascending aorta, Familial thoracic aortic aneurysm and aortic dissection, Loeys-Dietz syndrome
Health Risk
RS112465572
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Loeys-Dietz syndrome 2, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS1212599579
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Diabetic retinopathy, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS1226482581
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS1247177623
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, TGFBR2-related disorder, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS1269699495
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Diabetic retinopathy, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
All Variants (234)
RSID Category Clinical Significance Conditions
RS878854612 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Loeys-Dietz syndrome 2, TGFBR2-related disorder
RS886058297 Health Risk Conflicting classifications of pathogenicity Loeys-Dietz syndrome 2, Malignant tumor of esophagus, Colorectal cancer
RS952006496 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Loeys-Dietz syndrome 2, Diabetic retinopathy
RS984098699 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Diabetic retinopathy, Loeys-Dietz syndrome 2
RS104893810 Health Risk Likely pathogenic Loeys-Dietz syndrome 2, Familial thoracic aortic aneurysm and aortic dissection, Loeys-Dietz syndrome
RS1060501984 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS113786762 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS121918714 Health Risk Likely pathogenic Malignant tumor of esophagus, Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS1553630174 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS1553630221 Health Risk Likely pathogenic Inborn genetic diseases, Inborn genetic diseases
RS1553630274 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS1553630289 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS1553630426 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Loeys-Dietz syndrome 2, Familial thoracic aortic aneurysm and aortic dissection
RS1553630438 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS1559472349 Health Risk Likely pathogenic Cardiovascular phenotype, Familial thoracic aortic aneurysm and aortic dissection, Cardiovascular phenotype
RS1575165264 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS1575165272 Health Risk Likely pathogenic Loeys-Dietz syndrome 1, Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS1699354286 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS1699361288 Health Risk Likely pathogenic
RS1699392675 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS1699708963 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS1699709401 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS193922664 Health Risk Likely pathogenic Loeys-Dietz syndrome, Loeys-Dietz syndrome
RS2125434732 Health Risk Likely pathogenic Loeys-Dietz syndrome 2, Loeys-Dietz syndrome 2
RS2125436184 Health Risk Likely pathogenic Loeys-Dietz syndrome 2, Loeys-Dietz syndrome 2
RS2125436283 Health Risk Likely pathogenic
RS2125436704 Health Risk Likely pathogenic Isolated thoracic aortic aneurysm, Familial thoracic aortic aneurysm and aortic dissection, Loeys-Dietz syndrome 2
RS2125436754 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Loeys-Dietz syndrome 2, Loeys-Dietz syndrome 2
RS2125436781 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS2125436797 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS2125439099 Health Risk Likely pathogenic Loeys-Dietz syndrome 2, Loeys-Dietz syndrome 2
RS2125455181 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS2125455206 Health Risk Likely pathogenic Loeys-Dietz syndrome 2, Loeys-Dietz syndrome 2
RS2125455285 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS2125455358 Health Risk Likely pathogenic Loeys-Dietz syndrome 2, Loeys-Dietz syndrome 2
RS2125455385 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS2125455389 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS2125455394 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS2470563136 Health Risk Likely pathogenic
RS2470564348 Health Risk Likely pathogenic Loeys-Dietz syndrome 2, Loeys-Dietz syndrome 2
RS2470568337 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, TGFBR2-related disorder, Familial thoracic aortic aneurysm and aortic dissection
RS397516840 Health Risk Likely pathogenic Loeys-Dietz syndrome, Loeys-Dietz syndrome
RS528566980 Health Risk Likely pathogenic Isolated thoracic aortic aneurysm, Isolated thoracic aortic aneurysm
RS587776770 Health Risk Likely pathogenic Loeys-Dietz syndrome 2, Loeys-Dietz syndrome 2
RS587782979 Health Risk Likely pathogenic Loeys-Dietz syndrome, Loeys-Dietz syndrome 2, Familial thoracic aortic aneurysm and aortic dissection
RS772490613 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS863223841 Health Risk Likely pathogenic
RS863223848 Health Risk Likely pathogenic
RS863223850 Health Risk Likely pathogenic
RS863223851 Health Risk Likely pathogenic
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