The UMD-THAP1 mutations database
Record ID: 166

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.109_132dupp.Glu37_Asn44dupHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TTTPheins24aInFIn frame insFr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
L3-THAP dom. DNA binding -

Mutation impact


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

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
SER01BEL F0003 I0001patient 4ProbandFemaleFamilialSERBIA

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomSeverityAge
Age at last examination38
Age of onsetneck12
Armmild38
Dysarthriamild38
Jawmild38
Larynxsevere38
Legmild38
NeckSevere38
Speech38
Trunkmoderate38

Comments


2 asymptomatic carriers in the family

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.