The UMD-FBN1 mutations database
Record ID: 695

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.2468delAp.Asn823ThrfsX24HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AACAsndel1bFs.Stop at 846Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #09 Ca2+ binding

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
AUS02WES F0009 I01ProbandNANA? (7 years old)AUSTRALIA

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Asc. aortic dilatation
S-Arachnodactyly (M)
S-Pectus carinatum (M)(2)
S-Plain pes planus (M)(1)
S-Reduced US/LS ratio <0.87 (M)
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.