The UMD-THAP1 mutations database
Record ID: 147

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 I0001case 2ProbandFemaleFamilialCHINA

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination25
Age of onsetneck20
Faceblepharospasm25
Neck20
Trunk25

Comments


RNA expression level: 0.97 +/- 0.12

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.