The UMD-THAP1 mutations database
Record ID: 21

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.17C>Tp.Ser6PheHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TCCSerTTCPheC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
L1-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)
0.710.06 (non pathogenous)82 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
UKD02LON F0002 I0001-Family EProbandFemaleFamilialGERMANY

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomSeverityAge
Age at last examination50
Age of onsethand20
Arm50
Incomplete clinical description
Neck

Comments


Incomplete clinical description

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.