The UMD-THAP1 mutations database
Record ID: 24

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.77C>Gp.Pro26ArgHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CCTProCGTArgC->GTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
L2-THAP dom. DNA binding -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)
0.790.02 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
UKD02LON F0004 I0001Family MProbandFemaleFamilialU.K.

Phenotypic groupDisease
NAFocal dystonia

Clinical data


SymptomSeverityAge
Age at last examination56
Age of onsetface17
Face17
Jaw17
Speech56

Comments


Patient presenting also with variation c.72-4T>C (This mutation has probably no impact on splicing).

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.