The UMD-THAP1 mutations database
Record ID: 177

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.46A>Gp.Lys16GluHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AAGLysGAGGluA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
L1-THAP dom. Yes, non 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)
0.710.09 (non pathogenous)47 (Polymorphism)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
USA02MEN F0011 I0001ProbandMaleFamilialU.S.A.

Phenotypic groupDisease
NAGeneralized dystonia

Clinical data


SymptomSeverityAge
Age at last examination44
Age of onsetlarynx6
Armbilateral44
FaceUpper face44
FaceLower face44
Jaw44
Larynx44
Legbilateral44
Neck44
Tongue44
Trunk44

Reference


Reference IDPubMed IDReference
2522377579
LeDoux MS, Xiao J, Rudzinska M, Bastian RW, Wszolek ZK, Van Gerpen JA, Puschmann A, Momcilovic D, Vemula SR, Zhao Y. Genotype-phenotype correlations in THAP1 dystonia: molecular foundations and description of new cases.Parkinsonism Relat Disord. 2012 Jun;18(5):414-25.