The UMD-THAP1 mutations database
Record ID: 146

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.63_66delTTTCp.Phe22ThrfsX50HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TCTSerdel4cFs.Stop at 71Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
L1-THAP dom. 

Mutation impact


At the mRNA levelOn restriction map
Deletion of a repeated sequenceNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
CHI01PEK F0003 F0001case 1ProbandMaleFamilialCHINA

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination21
Age of onsetarm12
Armhand tremor21
Dysarthria21
Neck21

Comments


RNA expression level: 1.30 +/- 0.11

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.