The UMD-THAP1 mutations database
Record ID: 153

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.57C>Tp.Pro19ProHeterozygousPolymorphism?

wt codonwt aamutant codonmutant aamutational eventmutation type
CCCProCCTProC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
L1-THAP dom. Yes, coding strandYes

Mutation impact


At the mRNA levelOn restriction map
Not tested on cDNA (needed)New restriction site(s): none
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.710.30 (non pathogenous)23 (Polymorphism)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
USA02MEN F0007 I0001ProbandFemaleUnknownU.S.A.

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination85
Age of onsetlarynx33
blepharospasm85
Jaw85
Larynx33

Comments


Creation of an exonic ESS site. Potential alteration of splicing.

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.