The UMD-THAP1 mutations database
Record ID: 150

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.161G>Tp.Cys54PheHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGTCysTTTPheG->TTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
BS2-THAP dom. C2CH motifNoNo

Mutation impact


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

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.790.03 (pathogenous)98 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
CHI01PEK F0004 I0002Father of case 2RelativeMaleFamilialCHINA

Phenotypic groupDisease
NAFocal dystonia

Clinical data


SymptomAge
Age at last examination58
Age of onset50
Paroxysmal head shake50

Comments


Fu Bo Cheng (personal communication, 08/08/2011)

Reference


Reference IDPubMed IDReference
20 21800139
Cheng FB, Ozelius LJ, Wan XH, Feng JC, Ma LY, Yang YM, Wang L. THAP1/DYT6 sequence variants in non-DYT1 early-onset primary dystonia in China and their effects on RNA expression. J Neurol. 2012 Feb;259(2):342-7.