The UMD-FBN1 mutations database
Record ID: 107

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.4270C>Gp.Pro1424AlaHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CCAProGCAAlaC->GTv

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)
10.25 (non pathogenous)71 (Probably pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
AUS01NAD F0008 I01ProbandMalefamilial? (2,5 years old)AUSTRALIA

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


SymptomSeverity
O-Divergent strabismus
O-Myopiaearly
S-Arachnodactyly (M)
S-Characteristic facial appearance
S-Joint hypermobility (m)

Reference


Reference IDPubMed IDReference
11414695540
Biggin A, Holman K, Brett M, Bennetts B, Ades L. "Detection of thirty novel FBN1 mutations in patients with Marfan syndrome or a related fibrillinopathy". Hum Mutat. 2004 Jan;23(1):99.