The UMD-THAP1 mutations database
Record ID: 224

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 F0001 I0001III.73ProbandFemaleFamilialBRAZIL

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination25
Age of onsetarm18
Arm18
Jaw25
Neck25

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.