The UMD-THAP1 mutations database
Record ID: 191

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.496G>Ap.Ala166ThrHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GCAAlaACAThrG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
Coiled-Coil dom. THAP1 dimerizationNoYes

Mutation impact


At the mRNA levelOn restriction map
No mechanism suspectedNew restriction site(s): none
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.930.42 (non pathogenous)82 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
SER01BEL F0005 I0001patient 5ProbandMaleNo family historySERBIA

Phenotypic groupDisease
NAMultifocal dystonia

Clinical data


SymptomSeverityAge
Age at last examination24
Age of onsetarm7
Armmoderate7

Reference


Reference IDPubMed IDReference
2323180184
Dobricic V., Kresojevic N., Svetel M., Jankovic M., Petrovic I., Tomic AD., Novakovic I., Kostic V.S. Mutation screening of the DYT6/THAP1 gene in Serbian dystonia patients with primary dystonia. J Neurol 2013 Apr;260(4):1037-42.