The UMD-THAP1 mutations database
Record ID: 20

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.7C>Tp.Gln3XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CAGGlnTAGStopC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
L1-THAP dom. NoNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Rma I
Lost restriction site(s): Nla IV

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
UKD02LON F0001 I0001-Family FProbandFemaleNo family historyU.K.

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomSeverityAge
Age at last examination37
Age of onsetlarynx8
Incomplete clinical description
Larynx8
Speech37

Comments


Incomplete clinical description

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.