The UMD-THAP1 mutations database
Record ID: 195

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
USA02MEN F0009 I0002Son of USA02MEN F0009 I0001RelativeMaleFamilialU.S.A.

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination20
Age of onsetarm20
Arm20

Comments


Also described in ref 40 (son of patient 2).

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.