The UMD-THAP1 mutations database
Record ID: 164

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.62C>Gp.Ser21CysHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TCTSerTGTCysC->GTv

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

Mutation impact


At the mRNA levelOn restriction map
No mechanism suspectedNew restriction site(s): none
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.710.00 (pathogenous)65 (Probably pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
SER01BEL F0001 I0001patient 1ProbandMaleFamilialSERBIA

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomSeverityAge
Age at last examination25
Age of onsetneck12
Armmild14
Dysarthriamild25
Jawmild25
Legmild25
NeckSevere12
Tonguemild25

Comments


mother and sister are symptomatic carriers

Reference


Reference IDPubMed IDReference
2323180184
Dobricic V., Kresojevic N., Svetel M., Jankovic M., Petrovic I., Tomic AD., Novakovic I., Kostic V.S. Mutation screening of the DYT6/THAP1 gene in Serbian dystonia patients with primary dystonia. J Neurol 2013 Apr;260(4):1037-42.