The UMD-FBN1 mutations database
Record ID: 2136

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.7916A>Gp.Tyr2639CysHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TATTyrTGTCysA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #42 NoNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): none

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

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
NET05GRO F0001 I0001ProbandMalefamilial11 years oldNETHERLAND

Phenotypic groupDisease
NAClassical MFS

Clinical data


SymptomAge
C-Asc. aortic dilatation24
C-Mitral valve prolapse24
O-Ectopia lentis24
S-Arachnodactyly (M)24
S-Pectus carinatum (M)(2)24
S-Plain pes planus (M)(1)24
SI-Significant striae atrophicae (m)(1)24

Reference


Reference IDPubMed IDReference
19720200614
Aalberts JJ, Schuurman AG, Pals G, Hamel BJ, Bosman G, Hilhorst-Hofstee Y, Barge-Schaapveld DQ, Mulder BJ, van den Berg MP, van Tintelen JP. "Recurrent and founder mutations in the Netherlands: Extensive clinical variability in Marfan syndrome patients with a single novel recurrent fibrillin-1 missense mutation". Neth Heart J. 2010 Feb;18(2):85-9.