The UMD-THAP1 mutations database
Record ID: 180

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.389_390delCAp.Ser130CysfsX4HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TCASerdel2bFs.Stop at 133Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
 

Mutation impact


At the mRNA levelOn restriction map
No mechanism suspectedNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
JAP01TOK F0001 I0001IV-71ProbandMaleFamilialJAPAN

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomSeverityAge
Age at last examination34
Age of onsetspeech +arms +legs15
Arm15
Cognitive dysfunctionIQ82
Cranial25
Deep Brain Stimulation30
Face18
Jaw25
Larynx25
Leg15
Neck18
Speech15
Tongue25
Trunk25

Comments


Patient underwent thalamic VLa DBS (Mure et al. Stereotactic and functional neurosurgery, 2014, 92:393-396).

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.