The UMD-THAP1 mutations database
Record ID: 32

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.50A>Gp.Asp17GlyHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GACAspGGCGlyA->GTs

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

Mutation impact


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

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.710.05 (pathogenous)79 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
USA02MEN F0003 I0001D-1378-ProbandFemaleNo family historyU.S.A.

Phenotypic groupDisease
NAFocal dystonia

Clinical data


SymptomSeverityAge
Age at last examination56
Age of onsetneck43
Neck43

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.