TGFB3 Chromosome 14

Transforming growth factor beta 3
93 variants 93 Health Risk

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What This Gene Does
This gene encodes a secreted ligand of the TGF-beta (transforming growth factor-beta) superfamily of proteins. Ligands of this family bind various TGF-beta receptors leading to recruitment and activation of SMAD family transcription factors that regulate gene expression. The encoded preproprotein is proteolytically processed to generate a latency-associated peptide (LAP) and a mature peptide, and is found in either a latent form composed of a mature peptide homodimer, a LAP homodimer, and a latent TGF-beta binding protein, or in an active form consisting solely of the mature peptide homodimer. The mature peptide may also form heterodimers with other TGF-beta family members. This protein is involved in embryogenesis and cell differentiation, and may play a role in wound healing. Mutations in this gene are a cause of aortic aneurysms and dissections, as well as familial arrhythmogenic right ventricular dysplasia 1. [provided by RefSeq, Aug 2016]
Gene Info
Gene Group
Transforming growth factor beta family
Locus Type
gene with protein product
Location
14q24.3
Ensembl
ENSG00000119699
Associated Conditions (13)
Familial thoracic aortic aneurysm and aortic dissection
Rienhoff syndrome
Arrhythmogenic right ventricular cardiomyopathy
Hypertrophic cardiomyopathy
Long QT syndrome
TGFB3-related disorder
Arrhythmogenic right ventricular dysplasia 1
Cardiomyopathy
Hereditary disorder of connective tissue
TGFB3-related connective tissue disorders
Primary dilated cardiomyopathy
Loeys-Dietz syndrome
Developmental disorder
Key Variants
RS1221353422
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS1414628117
Conflicting classifications of pathogenicity
Rienhoff syndrome, Rienhoff syndrome
Health Risk
RS142047577
Conflicting classifications of pathogenicity
Arrhythmogenic right ventricular cardiomyopathy, Hypertrophic cardiomyopathy, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS142069844
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS142601521
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Arrhythmogenic right ventricular dysplasia 1, Rienhoff syndrome
Health Risk
RS143229915
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Cardiomyopathy, Rienhoff syndrome
Health Risk
RS1555360212
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Hereditary disorder of connective tissue
Health Risk
RS1594792447
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, TGFB3-related disorder
Health Risk
RS1595337739
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS199791687
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Arrhythmogenic right ventricular dysplasia 1
Health Risk
RS201453600
Conflicting classifications of pathogenicity
Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome
Health Risk
RS201637508
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
All Variants (93)
RSID Category Clinical Significance Conditions
RS1221353422 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS1414628117 Health Risk Conflicting classifications of pathogenicity Rienhoff syndrome, Rienhoff syndrome
RS142047577 Health Risk Conflicting classifications of pathogenicity Arrhythmogenic right ventricular cardiomyopathy, Hypertrophic cardiomyopathy, Familial thoracic aortic aneurysm and aortic dissection
RS142069844 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS142601521 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Arrhythmogenic right ventricular dysplasia 1, Rienhoff syndrome
RS143229915 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Cardiomyopathy, Rienhoff syndrome
RS1555360212 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Hereditary disorder of connective tissue
RS1594792447 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, TGFB3-related disorder
RS1595337739 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS199791687 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Arrhythmogenic right ventricular dysplasia 1
RS201453600 Health Risk Conflicting classifications of pathogenicity Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome
RS201637508 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS2035184283 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS2035294180 Health Risk Conflicting classifications of pathogenicity TGFB3-related connective tissue disorders, Rienhoff syndrome, TGFB3-related connective tissue disorders
RS2140236263 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS369281541 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS369435862 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Arrhythmogenic right ventricular dysplasia 1, Rienhoff syndrome
RS374423962 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS375794497 Health Risk Conflicting classifications of pathogenicity Rienhoff syndrome, Rienhoff syndrome
RS375846390 Health Risk Conflicting classifications of pathogenicity
RS3917215 Health Risk Conflicting classifications of pathogenicity
RS754211381 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS757664433 Health Risk Conflicting classifications of pathogenicity Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome
RS762643638 Health Risk Conflicting classifications of pathogenicity Rienhoff syndrome, Arrhythmogenic right ventricular dysplasia 1, Familial thoracic aortic aneurysm and aortic dissection
RS763289805 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, TGFB3-related disorder
RS766164843 Health Risk Conflicting classifications of pathogenicity Primary dilated cardiomyopathy, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS768188445 Health Risk Conflicting classifications of pathogenicity Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome
RS769627183 Health Risk Conflicting classifications of pathogenicity Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome
RS779502039 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS779897294 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS780891440 Health Risk Conflicting classifications of pathogenicity Rienhoff syndrome, Rienhoff syndrome
RS781353815 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Arrhythmogenic right ventricular dysplasia 1
RS868258653 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Arrhythmogenic right ventricular dysplasia 1, Rienhoff syndrome
RS920721092 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Arrhythmogenic right ventricular dysplasia 1
RS921588962 Health Risk Conflicting classifications of pathogenicity Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS1057522944 Health Risk Likely pathogenic
RS1060502826 Health Risk Likely pathogenic Rienhoff syndrome, Loeys-Dietz syndrome, Familial thoracic aortic aneurysm and aortic dissection
RS1555360027 Health Risk Likely pathogenic Rienhoff syndrome, Rienhoff syndrome
RS1555360362 Health Risk Likely pathogenic Rienhoff syndrome, Rienhoff syndrome
RS1555360883 Health Risk Likely pathogenic Rienhoff syndrome, Rienhoff syndrome
RS1566679646 Health Risk Likely pathogenic Rienhoff syndrome, Rienhoff syndrome
RS1566682530 Health Risk Likely pathogenic Rienhoff syndrome, Rienhoff syndrome
RS1594787989 Health Risk Likely pathogenic
RS2035180096 Health Risk Likely pathogenic Rienhoff syndrome, Familial thoracic aortic aneurysm and aortic dissection, Rienhoff syndrome
RS2035287906 Health Risk Likely pathogenic Rienhoff syndrome, Arrhythmogenic right ventricular dysplasia 1, Rienhoff syndrome
RS2140238043 Health Risk Likely pathogenic
RS2503023443 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS2503023741 Health Risk Likely pathogenic TGFB3-related disorder, TGFB3-related disorder
RS2503023915 Health Risk Likely pathogenic TGFB3-related disorder, TGFB3-related disorder
RS2504093512 Health Risk Likely pathogenic Rienhoff syndrome, Rienhoff syndrome
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