The UMD-THAP1 mutations database
Record ID: 51

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.449A>Cp.His150ProHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CATHisCCTProA->CTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
NLS NoNo

Mutation impact


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

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.930.24 (non pathogenous)76 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
CHI01PEK F0002 I0001I-4986ProbandMaleNo family historyCHINA

Phenotypic groupDisease
NAMultifocal dystonia

Clinical data


SymptomSeverityAge
Age at last examination42
Age of onsetlarynx22
Larynx22
Neck42

Comments


RNA expression level: 1.04 +/- 0.16 (ref Cheng et al. J Neurol 2011)

Reference


Reference IDPubMed IDReference
1320825472
Cheng FB, Wan XH, Feng JC, Wang L, Yang YM, Cui LY. Clinical and genetic evaluation of DYT1 and DYT6 primary dystonia in China. Eur J Neurol. 2011 Mar;18(3):497-503.