The UMD-THAP1 mutations database
Record ID: 27

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.236delCp.Thr79LysfsX41HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ACAThrdel1bFs.Stop at 119Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
H4-THAP dom. AVPTIF motif

Mutation impact


At the mRNA levelOn restriction map
No mechanism suspectedNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
UKD02LON F0007 I0001-Family LHProbandFemaleFamilialU.K.

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination29
Age of onsetarm10
Armbilateral10
Neck29
Speech29

Reference


Reference IDPubMed IDReference
820211909
Houlden H, Schneider SA, Paudel R, Melchers A, Schwingenschuh P, Edwards M, Hardy J, Bhatia KP. THAP1 mutations (DYT6) are an additional cause of early-onset dystonia. Neurology. 2010 Mar 9;74(10):846-50.