The UMD-THAP1 mutations database
Record ID: 148

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.267G>Ap.Lys89LysHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AAGLysAAALysG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
 Yes, non coding strandNo

Mutation impact


At the mRNA levelOn restriction map
Last nucleotide of the exon, skipping of the exon 2New restriction site(s): none
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.791.00 (non pathogenous)71 (Probably pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
CHI01PEK F0005 I0001case 4ProbandMaleFamilialCHINA

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination29
Age of onsetneck21
Jaw29
Neck21

Comments


RNA expression level: 0.38 +/- 0.09

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.