The UMD-THAP1 mutations database
Record ID: 152

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.539T>Cp.Leu180SerHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TTALeuTCASerT->CTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
Coiled-Coil dom. Yes, coding strandNo

Mutation impact


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

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.930.03 (pathogenous)81 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
CHI01PEK F0006 I0001case 7ProbandMaleNo family historyCHINA

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination40
Age of onsetneck25
Dysarthria40
Neck25

Comments


RNA expression level: 1.23 +/- 0.05

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.