The UMD-THAP1 mutations database
Record ID: 151

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.267G>Ap.Lys89LysHeterozygousPolymorphism

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 I0002sister of case 4RelativeFemaleFamilialCHINA

Phenotypic groupDisease
NAFocal dystonia

Clinical data


SymptomAge
Age at last examination36
Age of onset30
Essential hand tremor30

Comments


Fu Bo Cheng (personal communication, 08/08/2011)

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.