The UMD-THAP1 mutations database
Record ID: 227

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.289C>Tp.Gln97XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CAGGlnTAGStopC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
Proline rich reg. NoNo

Mutation impact


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

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
BRA02CUR F0003 I0001C3ProbandMaleNo family historyBRAZIL

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomSeverityAge
Age at last examination36
Age of onsetarm22
Armbilateral36
Dysarthria36
Jaw36
Legbilateral36
Neck36
Trunk36

Reference


Reference IDPubMed IDReference
3924757586
Camargo CH, Camargos ST, Raskin S, Cardoso FE, Teive HA. DYT6 in Brazil: Genetic Assessment and Clinical Characteristics of Patients. Tremor Other Hyperkinet Mov (N Y). 2014 Apr 15;4:226.