The UMD-FBN1 mutations database
Record ID: 1668

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.6431A>Gp.Asn2144SerHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AATAsnAGTSerA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #32 Ca2+ bindingYes, non coding strandNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Csp6 I, Rsa I
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.830.00 (pathogenous)71 (Probably pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
JAP07TOK F0001 I0001ProbandFemaleNAJAPAN

Phenotypic groupDisease
NANA

Clinical data


SymptomSeverityAge
C-Asc. aortic dissectionsurgery42
C-Mitral valve prolapsesurgery46
S-Arachnodactyly (M)
S-Increased body length46

Reference


Reference IDPubMed IDReference
15118049824
Shiga M, Saito M, Hattori M, Torii C, Kosaki K, Kiyono T, Suda N. "Characteristic phenotype of immortalized periodontal cells isolated from a Marfan syndrome type I patient". Cell Tissue Res. 2008 Feb;331(2):461-72.