The UMD-THAP1 mutations database
Record ID: 46

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.427A>Gp.Met143ValHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ATGMetGTGValA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
Coiled-Coil dom. Yes, non coding strandNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): ApaL I, Bsp1286 I, HgiA I
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.931.00 (non pathogenous)35 (Polymorphism)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
GER02TUE F0005 I0001case 5ProbandFemaleNo family historyGERMANY

Phenotypic groupDisease
NAFocal dystonia

Clinical data


SymptomSeverityAge
Age at last examination
Age of onsetneck46
Neck46

Reference


Reference IDPubMed IDReference
1220669277
S*hn AS, Gl*ckle N, Doetzer AD, Deuschl G, Felbor U, Topka HR, Sch*ls L, Riess O, Bauer P, MŸller U, Grundmann K. Prevalence of THAP1 sequence variants in German patients with primary dystonia. Mov Disord. 2010 Sep 15;25(12):1982-6.