The UMD-THAP1 mutations database
Record ID: 231

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.539T>Cp.Leu180SerHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TTALeuTCASerT->CTs

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

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): Mse I

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.930.03 (pathogenous)81 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
CHI01PEK F0008 I0001ProbandMaleFamilialCHINA

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination49
Age of onsetneck23
Armbilateral49
Neck49
Speech49

Comments


This patient habors also a c.581A>T (p.Asp194Val) mutation in the TOR1A gene.

Reference


Reference IDPubMed IDReference
4124862462
Cheng FB, Feng JC, Ma LY, Miao J, Ott T, Wan XH, Grundmann K. Combined occurrence of a novel TOR1A and a THAP1 mutation in primary dystonia. Mov Disord. 2014 Jul;29(8):1079-83.