The UMD-MYH11 mutations database
Record ID: 33

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3824G>Tp.Arg1275LeuHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGGArgCTGLeuG->TTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
Coiled coil Yes, non coding strandNo

Mutation impact


At the mRNA levelOn restriction map
New restriction site(s): BstN I, EcoR II
Lost restriction site(s): Bcn I, Bcn I, BstK I, Dsa V, Hpa II, Msp I, Nci I, Nci I, ScrF I, Sma I, Xma I

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.930.82 (non pathogenous)82 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA02HOU F0001 I0003RelativeMaleFamilialU.S.A.

Phenotypic groupDisease
Thoracic Aortic Aneurysm or Dissection

Clinical data


SymptomSeverityAge
C-Asc aortic dilatationSurgery22

Reference


Reference IDPubMed IDReference
217666408
Pannu H, Tran-Fadulu V, Papke CL, Scherer S, Liu Y, Presley C, Guo D, Estrera AL, Safi HJ, Brasier AR, Vick GW, Marian AJ, Raman CS, Buja LM, Milewicz DM. MYH11 mutations result in a distinct vascular pathology driven by insulin-like growth factor 1 and angiotensin II. Hum Mol Genet. 2007 Oct 15;16(20):2453-62. Epub 2007 Jul 31. .