The UMD-THAP1 mutations database
Record ID: 228

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.238A>Gp.Ile80ValHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ATAIleGTAValA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
H4-THAP dom. AVPTIF motifYes, non coding strandNo

Mutation impact


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

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.790.60 (non pathogenous)53 (Probable polymorphism)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
POL02LOD F0002 I00011ProbandMaleFamilialPOLAND

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomSeverityAge
Age at last examination29
Age of onsethand7
Armbilateral29
Larynx29
Legbilateral29
Neck29

Reference


Reference IDPubMed IDReference
4026087139
Golanska E, Gajos A, Sieruta M, Szybka M, Rudzinska M, Ochudlo S, Kmiec T, Liberski PP, Bogucki A. Screening for THAP1 Mutations in Polish Patients with Dystonia Shows Known and Novel Substitutions. PLoS One. 2015 Jun 18;10(6):e0129656.