The UMD-THAP1 mutations database
Record ID: 192

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.208A>Gp.Lys70GluHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AAGLysGAGGluA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
L4-THAP dom. Yes, non 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)
0.790.03 (pathogenous)51 (Probable polymorphism)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
GRE01LAR F0001 I0001ProbandFemaleNo family historyGREECE

Phenotypic groupDisease
NABlepharospasm

Clinical data


SymptomSeverityAge
Age at last examination64
Age of onseteyes55
blepharospasm55

Reference


Reference IDPubMed IDReference
3222903657
Xiromerisiou G, Houlden H, Scarmeas N, Stamelou M, Kara E, Hardy J, Lees AJ, Korlipara P, Limousin P, Paudel R, Hadjigeorgiou GM, Bhatia KP. THAP1 mutations and dystonia phenotypes: genotype phenotype correlations. Mov Disord. 2012 Sep 1;27(10):1290-4.