The UMD-FBN1 mutations database
Record ID: 308

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.4179_4187delp.Gly1394_Thr1396delHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GAAGludel9cInFIn frame delInF

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #19 

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
AUS01NAD F0018 I79ProbandMalede novofirst year of life (3 years old)AUSTRALIA

Phenotypic groupDisease
NANeonatal MFS

Clinical data


Symptom
C-Mitral regurgitation
C-Mitral regurgitation
C-Mitral valve prolapse
O-Ectopia lentis
O-Iridodonesis

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.