The UMD-FBN1 mutations database
Record ID: 160

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.2954G>Ap.Gly985GluHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GGGGlyGAGGluG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP#03 Yes, non coding strandNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Taq 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
FRA01BOU F0115 I0153ProbandMaleparental mosaicism? (16 years old)FRANCE

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Asc. aortic dissection
C-Mitral regurgitation
C-Mitral valve prolapse
S-Arachnodactyly (M)
S-High arched palate
S-Increased body length
S-Scoliosis > 20° (M)(1)

Reference


Reference IDPubMed IDReference
6210441597
Collod-Beroud G, Lackmy-Port-Lys M, Jondeau G, Mathieu M, Maingourd Y, Coulon M, Guillotel M, Junien C, Boileau C. "Demonstration of the recurrence of Marfan-like skeletal and cardiovascular manifestations due to germline mosaicism for an FBN1 mutation". Am J Hum Genet 1999 Sep;65(3):917-21.