The UMD-THAP1 mutations database
Record ID: 204

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.83C>Tp.Thr28IleHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ACTThrATTIleC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
L2-THAP dom. Yes, coding strandNo

Mutation impact


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

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.790.13 (non pathogenous)65 (Probably pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
BRA01SAO F0006 I0002Sib of patient E.1-RelativeMaleFamilialBRAZIL

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination16
Age of onsetneck + trunk14
Neck14
Trunk14

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.