The UMD-FBN1 mutations database
Record ID: 2836

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.7456_7821delp.Leu2486_Asp2607delHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CTTLeudel366aInFIn frame delInF

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #39 Ca2+ binding

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 F0001 I06RelativeFemalefamilialU.K.

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom

Reference


Reference IDPubMed IDReference
2097945217
Raghunath M, Kielty CM, Kainulainen K, Child A, Peltonen L, Steinmann B. "Analyses of truncated fibrillin caused by a 366 bp deletion in the FBN1 gene resulting in Marfan syndrome". Biochem J. 1994;302 (Pt 3):889-96.