The UMD-THAP1 mutations database
Record ID: 217

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.167A>Gp.Glu56GlyHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GAGGluGGGGlyA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
H2-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)
0.790.09 (non pathogenous)76 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
POL02LOD F0001 I0001IV.3ProbandMaleFamilialPOLAND

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomSeverityAge
Age at last examination56
Age of onsetspeech10
Armbilateral10
blepharospasm53
Footbilateral53
Neck50
Speech10
Trunk53

Comments


Also described in ref 40.

Reference


Reference IDPubMed IDReference
3725385508
Gajos A, Golanska E, Sieruta M, Szybka M, Liberski PP, Bogucki A. High variability of clinical symptoms in a Polish family with a novel THAP1 mutation. Int J Neurosci. 2014 Nov 11. [Epub ahead of print]