The UMD-THAP1 mutations database
Record ID: 196

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
BRA01SAO F0002 I0001A.1-ProbandMaleFamilialBRAZIL

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination17
Age of onsetarm3
Arm3
Neck17
Trunk17

Reference


Reference IDPubMed IDReference
3524976531
da Silva-Junior FP, dos Santos CO, Silva SM, Barbosa ER, Borges V, Ferraz HB, Limongi JC, Rocha MS, de Carvalho Aguiar P. Novel THAP1 variants in Brazilian patients with idiopathic isolated dystonia. J Neurol Sci. 2014 Sep 15;344(1-2):190-2.