The UMD-THAP1 mutations database
Record ID: 26

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.174delTp.Phe58LeufsX15HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TTTPhedel1cFs.Stop at 72Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
THAP dom. DNA binding -

Mutation impact


At the mRNA levelOn restriction map
Deletion in a stretch of nucleotideNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
UKD02LON F0006 I0001-Family KProbandFemaleNo family historyGREECE

Phenotypic groupDisease
NAFocal dystonia

Clinical data


SymptomSeverityAge
Age at last examination38
Age of onsetarm+cranial20
Arm20
Cranial20
Jaw38
Speech38
Tonguesevere38

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.