The UMD-THAP1 mutations database
Record ID: 58

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1A>Gp.Met1?HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ATGMetGTGValA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
THAP dom. NoNo

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)
0.710.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
BRA01SAO F0001 I0002patient 14's brother-RelativeMaleFamilialBRAZIL

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomSeverityAge
Age at last examination31
Age of onsetarm6
Armbilateral6
Larynx31
Legbilateral21
Neck31
Speech21
Trunk31

Comments


Described as patient G.1 in ref 35.

Reference


Reference IDPubMed IDReference
1420925076
De Carvalho Aguiar P, Fuchs T, Borges V, Lamar KM, Silva SM, Ferraz HB, Ozelius L. Screening of Brazilian families with primary dystonia reveals a novel THAP1 mutation and a de novo TOR1A GAG deletion. Mov Disord. 2010 Dec 15;25(16):2854-7.