The UMD-FBN1 mutations database
Record ID: 208

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.4704delAp.Ala1569ProfsX12HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AAALysdel1cFs.Stop at 1580Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP#04 

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
AUS01NAD F0009 I01ProbandMalefamilialat 11 years old23 years oldAUSTRALIA

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Asc. aortic dilatation
C-Mitral valve prolapse
O-Increased axial length of globe (m)
S-Arachnodactyly (M)
S-Characteristic facial appearance
S-Chest deformity (unspecified)
S-High arched palate
S-Joint hypermobility (m)
S-Plain pes planus (M)(1)
S-Protusio acetabulæ (M)(2)
SI-Significant striae atrophicae (m)(1)

Reference


Reference IDPubMed IDReference
569746367
Gibson MA, Ellis SL, Ades LC, Haan E, Cleary EG. "Preferential pre-mRNA utilisation of an upstream cryptic 5' splice site created by a single base deletion mutation in exon 37 of the FBN-1 gene". Eur J Biochem 1998 Aug 15;256(1):221-8.