The UMD-FBN1 mutations database
Record ID: 2849

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.8547T>Gp.Tyr2849XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TATTyrTAGStopT->GTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
FibuCTDIII-like motif NoNo

Mutation impact


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

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
CHI08BEI F0002 I0002RelativeMalefamilialCHINA

Phenotypic groupDisease
NAMFS

Clinical data


SymptomAge
L-Spontaneous pneumothorax26
S-Arachnodactyly (M)26
S-Increased body length26
S-Joint hypermobility (m)26

Reference


Reference IDPubMed IDReference
21721034599
Gao LG, Zhang L, Song L, Wang H, Chang Q, Wu YB, Hui RT, Zhou XL. "Identification of a novel lethal fibrillin-1 gene mutation in a Chinese Marfan family and correlation of 3' fibrillin-1 gene mutations with phenotype". Chin Med J (Engl). 2010 Oct;123(20):2874-8.