The UMD-TGFBR2 mutations database
Record ID: 210

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1483C>Tp.Arg495XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGAArgTGAStopC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #03 Yes, coding strandYes

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): Dpn I, Dpn II, Mbo I, Sau3A I, Taq I

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
JAP06SUI P0002 I0001 ProbandMalefamilial34 years old (35 years old)JAPAN

Phenotypic groupDisease
NALDS type I

Clinical data


Symptom
C-Arterial tortuosity
C-Asc. aortic dissection
CF-Exotropia
S-Pectus excavatum

Reference


Reference IDPubMed IDReference
3317652900
Akutsu K, Morisaki H, Takeshita S, Sakamoto S, Tamori Y, Yoshimuta T, Yokoyama N, Nonogi H, Ogino H, Morisaki T. "Phenotypic heterogeneity of Marfan-like connective tissue disorders associated with mutations in the transforming growth factor-beta receptor genes". Circ J. 2007 Aug;71(8):1305-9.