The UMD-THAP1 mutations database
Record ID: 221

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.413delCp.Thr138MetfsX15HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ACTThrdel1bFs.Stop at 152Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
 

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
JAP01TOK F0002 I00012ProbandFemaleFamilialJAPAN

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomSeverityAge
Age at last examination61
Age of onsethand9
Arm9
Legbilateral39
Neck39
Trunk39

Reference


Reference IDPubMed IDReference
3824227593
Miyamoto R, Koizumi H, Morino H, Kawarai T, Maruyama H, Mukai Y, Miyashiro A, Sako W, Izumi Y, Kawakami H, Kaji R. DYT6 in Japan-genetic screening and clinical characteristics of the patients. Mov Disord. 2014 Feb;29(2):278-80.