The UMD-THAP1 mutations database
Record ID: 37

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
UKD02LON F0009 I0001-Family JProbandFemaleNo family historyU.K.

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomSeverityAge
Age at last examination30
Age of onsetfoot3
Armbilateral30
Legbilateral30
Neck30
Speech30

Reference


Reference IDPubMed IDReference
820211909
Houlden H, Schneider SA, Paudel R, Melchers A, Schwingenschuh P, Edwards M, Hardy J, Bhatia KP. THAP1 mutations (DYT6) are an additional cause of early-onset dystonia. Neurology. 2010 Mar 9;74(10):846-50.