The UMD-THAP1 mutations database
Record ID: 199

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.109dupp.Glu37GlyfsX10HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GAGGluins1bFs.Stop at 46Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
AH1-THAP dom. DNA binding -

Mutation impact


At the mRNA levelOn restriction map
Duplication in a stretch of nucleotidesNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
BRA01SAO F0003 I0002Daughter of patient B.1-RelativeFemaleFamilialBRAZIL

Phenotypic groupDisease
NAHemidystonia

Clinical data


SymptomSeverityAge
Age at last examination9
Age of onsetleg3
Arm9
Leg3

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.