The UMD-FBN1 mutations database
Record ID: 37

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.364C>Tp.Arg122CysHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGCArgTGCCysC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
EGF-like #02 Yes, coding strandYes

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)
0.830.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
FIN01HEL F0014 I01ProbandMalefamilial? (21 years old)FINLAND

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


Symptom
O-Ectopia lentis
O-Spherophakia
S-Joint dislocation

Reference


Reference IDPubMed IDReference
188040326
Stahl-Hallengren C, Ukkonen T, Kainulainen K, Kristofersson U, Saxne T, Tornqvist K, Peltonen L. "An extra cysteine in one of the non-calcium-binding epidermal growth factor-like motifs of the FBN1 polypeptide is connected to a novel variant of Marfan syndrome". J Clin Invest 1994 Aug;94(2):709-13.