The UMD-THAP1 mutations database
Record ID: 154

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS1+9C>A (c.71+9C>A)HeterozygousPolymorphism

wt codonwt aamutant codonmutant aamutational eventmutation type
AAGLysspl+9Spl.C->ATv

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 (%)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
USA02MEN F0008 I0001ProbandFemaleUnknownU.S.A.

Phenotypic groupDisease
NAFocal dystonia

Clinical data


Symptom

Comments


No significant splicing motif alteration detected. This mutation has probably no impact on splicing. Variation also described in 7 other probands.

Reference


Reference IDPubMed IDReference
720083799
Xiao J, Zhao Y, Bastian RW, Perlmutter JS, Racette BA, Tabbal SD, Karimi M, Paniello RC, Wszolek ZK, Uitti RJ, Van Gerpen JA, Simon DK, Tarsy D, Hedera P, Truong DD, Frei KP, Dev Batish S, Blitzer A, Pfeiffer RF, Gong S, LeDoux MS. Novel THAP1 sequence variants in primary dystonia. Neurology. 2010 Jan 19;74(3):229-38.