The UMD-THAP1 mutations database
Record ID: 189

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.388_389delTCp.Val131PhefsX3HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TCASerdel2aFs.Stop at 133Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
 

Mutation impact


At the mRNA levelOn restriction map
Deletion of a repeated sequenceNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
JAP01TOK F0001 I0010IV-31RelativeMaleFamilialJAPAN

Phenotypic groupDisease
Reduced nuclear importAsymptomatic

Clinical data


Symptom

Comments


Reduced nuclear import demonstrated in ref 33.

Reference


Reference IDPubMed IDReference
31 22821615 228
Miyamoto R, Ohta E, Kawarai T, Koizumi H, Sako W, Izumi Y, Obata F, Kaji R. Broad spectrum of dystonia associated with a novel thanatosis-associated protein domain-containing apoptosis-associated protein 1 mutation in a Japanese family with dystonia 6, torsion. Mov Disord. 2012 Sep 1;27(10):1324-5.