The UMD-THAP1 mutations database
Record ID: 155

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.339T>Cp.Asp113AspHeterozygousPolymorphism

wt codonwt aamutant codonmutant aamutational eventmutation type
GATAspGACAspT->CTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
 NoNo

Mutation impact


At the mRNA levelOn restriction map
RNA expression not affectedNew restriction site(s): Hinc II
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.930.93 (non pathogenous)18 (Polymorphism)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
CHI01PEK F0007 I0001ProbandFemaleNo family historyCHINA

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination17
Age of onsetneck17
Neck17
Trunk17

Comments


Alteration of an exonic ESE site but no alteration of splicing. Asymptomatic mother with the same variation.

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.