The UMD-THAP1 mutations database
Record ID: 226

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.506G>Ap.Arg169GlnHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGAArgCAAGlnG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
Coiled-Coil dom. Yes, non coding strandYes

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)
0.860.18 (non pathogenous)47 (Polymorphism)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
BRA02CUR F0001 I0002III.23RelativeFemaleFamilialBRAZIL

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomSeverityAge
Age at last examination16
Age of onsetarm6
Armbilateral16
Dysarthria16
Jaw16
Larynx16
Legbilateral16
Neck16
Speech16
Trunk16

Comments


Double mutant: patient presented also a p.Arg169Gln mutation.

Reference


Reference IDPubMed IDReference
3924757586
Camargo CH, Camargos ST, Raskin S, Cardoso FE, Teive HA. DYT6 in Brazil: Genetic Assessment and Clinical Characteristics of Patients. Tremor Other Hyperkinet Mov (N Y). 2014 Apr 15;4:226.