The UMD-THAP1 mutations database
Record ID: 145

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS1-4T>C (c.72-4T>C)HeterozygousPolymorphism

wt codonwt aamutant codonmutant aamutational eventmutation type
AAGLysspl-4Spl.T->CTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
BS1-THAP dom. DNA binding -

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): none

Impact on splicing
Splice site type Wild type sequence CV Mutant type sequence CV Variation (%)
Acceptor
ccttcttcttagGT
86.6 _
ccttcttcctagGT
87.3 _
0.8 %

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
UKD02LON F0004 I0001Family MProbandFemaleFamilialU.K.

Phenotypic groupDisease
NAFocal dystonia

Clinical data


SymptomAge
Age at last examination56
Age of onset17
Face17
Jaw17
Speech56

Comments


No significant splicing motif alteration detected. This mutation has probably no impact on splicing. Patient presenting with the c.77C>G mutation.

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.