The UMD-FBN1 mutations database
Record ID: 128

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1421_1468delp.Cys474_Asp490delinsTyrHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGTCysdel48bInFIn frame delInF

StructureKey Residue (HCD)Pyrimidin doubletCpG
EGF-like #04 Disulfide bonds 460-474 (C4)

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
FIN01HEL F0020 I01ProbandMalede novo? (22 years old)FINLAND

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Mitral valve prolapse
C-Tricuspid valve prolapse
O-Ectopia lentis
O-Myopia
S-Arachnodactyly (M)
S-High arched palate
S-Joint hypermobility (m)
S-Scoliosis > 20° (M)(1)
SI-Inguinal hernia
SI-Significant striae atrophicae (m)(1)

Reference


Reference IDPubMed IDReference
68-
Rantamaki T, Peltonnen L (Personal communication 2000).