The UMD-FBN1 mutations database
Record ID: 309

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.4930C>Tp.Arg1644XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGAArgTGAStopC->TTs

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

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Nla III
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
AUS01NAD F0020 I08ProbandFemalede novoat 13 years oldAUSTRALIA

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Asc. aortic dilatation
C-Asc. aortic dilatation
O-Coloboma
S-Arachnodactyly (M)
S-Characteristic facial appearance
S-Crowding teeth (m)
S-High arched palate
S-Joint limitations
SI-Significant striae atrophicae (m)(1)

Reference


Reference IDPubMed IDReference
11414695540
Biggin A, Holman K, Brett M, Bennetts B, Ades L. "Detection of thirty novel FBN1 mutations in patients with Marfan syndrome or a related fibrillinopathy". Hum Mutat. 2004 Jan;23(1):99.