The UMD-THAP1 mutations database
Record ID: 194

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.62C>Gp.Ser21CysHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TCTSerTGTCysC->GTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
L1-THAP dom. Yes, 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)
0.710.00 (pathogenous)65 (Probably pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
USA02MEN F0010 I0002sister of USA02MEN F0010 I0001RelativeFemaleFamilialNA

Phenotypic groupDisease
NAHemidystonia

Clinical data


SymptomSeverityAge
Age at last examination26
Age of onsethand7
Arm26
Leg26

Reference


Reference IDPubMed IDReference
2522377579
LeDoux MS, Xiao J, Rudzinska M, Bastian RW, Wszolek ZK, Van Gerpen JA, Puschmann A, Momcilovic D, Vemula SR, Zhao Y. Genotype-phenotype correlations in THAP1 dystonia: molecular foundations and description of new cases.Parkinsonism Relat Disord. 2012 Jun;18(5):414-25.