The UMD-THAP1 mutations database
Record ID: 222

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.89C>Ap.Pro30HisHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CCCProCACHisC->ATv

StructureKey Residue (HCD)Pyrimidin doubletCpG
L2-THAP dom. Yes, 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)
0.790.01 (pathogenous)88 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
JAP01TOK F0003 I00013ProbandMaleFamilialJAPAN

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination51
Age of onsethand12
Hand12
Neck42

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.