The UMD-THAP1 mutations database
Record ID: 158

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.570delAp.Asp191ThrfsX9HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AAALysdel1cFs.Stop at 199Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
Coiled-Coil dom. 

Mutation impact


At the mRNA levelOn restriction map
Deletion in a stretch of nucleotideNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
GER01LUB F0006 I0001L3641ProbandFemaleFamilialGERMANY

Phenotypic groupDisease
NAFocal dystonia

Clinical data


SymptomSeverityAge
Age at last examination59
Age of onsetneck49
Neck49

Reference


Reference IDPubMed IDReference
22 21847143
Lohmann K, Uflacker N, Erogullari A, Lohnau T, Winkler S, Dendorfer A, Schneider SA, Osmanovic A, Svetel M, Ferbert A, Zittel S, KŸhn AA, Schmidt A, AltenmŸller E, MŸnchau A, Kamm C, Wittstock M, Kupsch A, Moro E, Volkmann J, Kostic V, Kaiser FJ, Klein C, BrŸggemann N. Identification and functional analysis of novel THAP1 mutations. Eur J Hum Genet. 2012 Feb;20(2):171-5.