The UMD-FBN1 mutations database
Record ID: 102

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1836delAp.Asp613ThrfsX12HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AAALysdel1cFs.Stop at 624Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #05 Ca2+ binding

Mutation impact


At the mRNA levelOn restriction map
Deletion in a stretch of nucleotidesNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
BRA01SPA F0001 I01ProbandMalefamilial? (6 years old)BRAZIL

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Asc. aortic dilatation
C-Mitral regurgitation
C-Mitral valve prolapse
O-Ectopia lentis
O-Myopia
S-Arachnodactyly (M)
S-Arm span/height >1.05 (M)
S-Chest deformity (unspecified)
S-Dolichostenomelia
S-Foot deformity
S-High arched palate
S-Joint hypermobility (m)
S-Long bone over growth
S-Reduced US/LS ratio <0.87 (M)
S-Scoliosis > 20° (M)(1)

Reference


Reference IDPubMed IDReference
3910189222
Perez AB, Pereira LV, Brunoni D, Zatz M, Passos-Bueno MR. "Identification of 8 new mutations in Brazilian families with Marfan syndrome". Mutations in brief no. 211. Online. Hum Mutat 1999;13(1):84.