The UMD-FBN1 mutations database
Record ID: 49

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.4857delAp.Gly1620GlufsX20HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GGAGlydel1cFs.Stop at 1639Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #23 

Mutation impact


At the mRNA levelOn restriction map
No mechanism suspectedNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA01BAL F0022 I01ProbandNAfamilialU.S.A

Phenotypic groupDisease
NAClassical MFS

Clinical data


SymptomSeverity
C-Asc. aortic dilatationsurgery
C-Mitral valve prolapse
O-Ectopia lentis
S-Long bone over growth
SI-Significant striae atrophicae (m)(1)

Reference


Reference IDPubMed IDReference
217611299
Nijbroek G, Sood S, McIntosh I, Francomano CA, Bull E, Pereira L, Ramirez F, Pyeritz RE, Dietz HC. "Fifteen novel FBN1 mutations causing Marfan syndrome detected by heteroduplex analysis of genomic amplicons". Am J Hum Genet 1995 Jul;57(1):8-21.