The UMD-THAP1 mutations database
Record ID: 43

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.410A>Gp.Tyr137CysHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TATTyrTGTCysA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
 HCF-1 binding NoNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Csp6 I, Rsa I
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
10.10 (non pathogenous)88 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
GER02TUE F0002 I0001Case 2-ProbandFemaleNo family historyGERMANY

Phenotypic groupDisease
NAFocal dystonia

Clinical data


SymptomSeverity
Age at last examination
Age of onsetjaw
Jaw
Larynx
Neck

Reference


Reference IDPubMed IDReference
1220669277
S*hn AS, Gl*ckle N, Doetzer AD, Deuschl G, Felbor U, Topka HR, Sch*ls L, Riess O, Bauer P, MŸller U, Grundmann K. Prevalence of THAP1 sequence variants in German patients with primary dystonia. Mov Disord. 2010 Sep 15;25(12):1982-6.