The UMD-THAP1 mutations database
Record ID: 162

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.68A>Cp.His23ProHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CACHisCCCProA->CTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
BS1-THAP dom. NoNo

Mutation impact


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

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.50.00 (pathogenous)88 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
GER01LUB F0005 I0001L4155ProbandMaleFamilialGERMANY

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination33
Age of onsetarm9
Arm9
Deep Brain Stimulation33

Comments


Mutation abolishs binding of THAP1 to TOR1A Same patient published in Lohmann et al. Eur J Hum Genet 2011

Reference


Reference IDPubMed IDReference
1920976771
Kaiser FJ, Osmanoric A, Rakovic A, Erogullari A, Uflacker N, Braunholz D, Lohnau T, Orolicki S, Albrecht M, Gillessen-Kaesbach G, Klein C, Lohmann K. The dystonia gene DYT1 is repressed by the transcription factor THAP1 (DYT6). Ann Neurol. 2010 Oct;68(4):554-9.