The UMD-FBN1 mutations database
Record ID: 706

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.7640delGp.Ser2547ThrfsX135HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AGCSerdel1bFs.Stop at 2681Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #40 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
AUS02WES F0020 I01ProbandNANA? (24 years old)AUSTRALIA

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Asc. aortic dilatation
O-Ectopia lentis
S-Characteristic facial appearance
S-Crowding teeth (m)
S-High arched palate
S-Joint hypermobility (m)
S-Pectus carinatum (M)(2)
S-Plain pes planus (M)(1)
S-Scoliosis > 20° (M)(1)
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.