The UMD-THAP1 mutations database
Record ID: 165

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.85C>Tp.Arg29XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGAArgTGAStopC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
L2-THAP dom. DNA binding -Yes, coding strandYes

Mutation impact


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

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
SER01BEL F0002 I0001patient 3ProbandMaleNo family historySERBIA

Phenotypic groupDisease
Reduced nuclear importSegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination40
Age of onsetlarynx36
Larynxmoderate36
Neckmoderate40

Comments


Reduced nuclear import demonstrated in ref 33.

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.