The UMD-THAP1 mutations database
Record ID: 25

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.150T>Gp.Tyr50XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TATTyrTAGStopT->GTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
L3-THAP dom. DNA binding -NoNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
UKD02LON F0005 I0001-Family GProbandFemaleNo family historyJEWISH

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination86
Age of onsetarm+head16
Arm16
Neck16
Speech86

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.