The UMD-THAP1 mutations database
Record ID: 169

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.86G>Ap.Arg29GlnHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGAArgCAAGlnG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
L2-THAP dom. DNA binding -Yes, non coding strandYes

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): Taq I, Tth111 I

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.790.06 (non pathogenous)65 (Probably pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
USA02MEN F0008 I0001ProbandMaleNo family historyU.S.A.

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination47
Age of onsetlarynx40
Armbilateral47
Face47
Jaw47
Larynx47
Neck47
Tongue47

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.