The UMD-FBN1 mutations database
Record ID: 530

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.4261C>Tp.Leu1421PheHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CTCLeuTTCPheC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #20 Ca2+ bindingYes, coding strandNo

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)
10.00 (pathogenous)71 (Probably pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
KOR01SEO F0003 I01ProbandMaleNA(47 years old)S. KOREA

Phenotypic groupDisease
NAClassical MFS

Clinical data


SymptomAge
CF-Dolichocephaly47
CNS-Lumbosacral dural ectasia47
S-Arachnodactyly (M)47
S-Joint limitations47

Reference


Reference IDPubMed IDReference
10011059536
Oh MR, Kim JS, Beck NS, Yoo HW, Lee HJ, Kohsaka T, Jin DK. "Six novel mutations of the fibrillin-1 gene in Korean patients with Marfan syndrome". Pediatr Int. 2000 Oct;42(5):488-91.