The UMD-THAP1 mutations database
Record ID: 18

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
UKD01LON F0002 I0001Case 2-ProbandMaleFamilialSPAIN

Phenotypic groupDisease
NAMultifocal dystonia

Clinical data


SymptomSeverityAge
Age at last examination33
Age of onsetarm14
Arm14
Foot33

Reference


Reference IDPubMed IDReference
519908320
Pais‡n-Ruiz C, Ruiz-Martinez J, Ruibal M, Mok KY, Indakoetxea B, Gorostidi A, Mass— JF. Identification of a novel THAP1 mutation at R29 amino-acid residue in sporadic patients with early-onset dystonia. Mov Disord. 2009 Dec 15;24(16):2428-9.