The UMD-THAP1 mutations database
Record ID: 216

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.15C>Gp.Cys5TrpHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGCCysTGGTrpC->GTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
L1-THAP dom. C2CH motifYes, coding strandNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Ava II, BsaJ I, Sty I
Lost restriction site(s): BspW I

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.710.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
POL01WAR F0001 I0003III.2RelativeMaleFamilialPOLANDf

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomAge
Age at last examination14
Age of onset4
Arm14
Jaw18
Leg14
Neck14
Speech14
Tongue18
Trunk18

Reference


Reference IDPubMed IDReference
3425168324
Jurek M, Hoffman-Zacharska D, Koziorowski D, M?dry J, Friedman A, Bal J. Intrafamilial variability of the primary dystonia DYT6 phenotype caused by p.Cys5Trp mutation in THAP1 gene. Neurol Neurochir Pol. 2014;48(4):254-7.