The UMD-FBN1 mutations database
Record ID: 368

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.7606G>Ap.Gly2536ArgHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GGGGlyAGGArgG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #40 Ca2+ bindingYes, non 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)
10.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD05LON F0005 I24ProbandFemalefamilial46 years oldU.K.

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


Symptom
C-Asc. aortic dilatation
C-Mitral regurgitation
C-Mitral valve prolapse
L-Pulmonary emphysema
O-Myopia
S-Arachnodactyly (M)
S-Arm span/height >1.05 (M)
S-Chondromalacia Patellae
S-Joint hypermobility (m)
S-Kyphosis
S-Plain pes planus (M)(1)
S-Reduced US/LS ratio <0.87 (M)
SI-Tendency to ecchymosis
SI-Varicose veins
SI-Varicose veins

Reference


Reference IDPubMed IDReference
8311748851
Comeglio P, Evans AL, Brice GW and Child AH. "Detection of six novel FBN1 mutations in british patients affected by Marfan syndrome". Human Mutation, 2001, 18(6): 546-7.