The UMD-FBN1 mutations database
Record ID: 141

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3095G>Ap.Cys1032TyrHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGCCysTACTyrG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #11 Disulfide bonds 1032-1044 (C1)NoNo

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)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD03DUN F0002 I01ProbandNAde novo?U.K.

Phenotypic groupDisease
NANeonatal MFS

Clinical data


Symptom
C-Congestive heart failure
C-Pulmonary art. dilatation
CF-Micrognathia
S-Arachnodactyly (M)
S-Camptodactyly
SI-Loose, redundant skin

Reference


Reference IDPubMed IDReference
4610404462
Ng DK, Chau KW, Black C, Thomas TM, Boxer M. "Neonatal Marfan syndrome: a case report". J Paediatr Child Health1999 Jun;35(3):321-3.