The UMD-THAP1 mutations database
Record ID: 168

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.20C>Ap.Ala7AspHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GCCAlaGACAspC->ATv

StructureKey Residue (HCD)Pyrimidin doubletCpG
L1-THAP dom. Yes, 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)
0.710.03 (pathogenous)92 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
USA02MEN F0007 I0001ProbandMaleNo family historyU.S.A.

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination24
Age of onsetlarynx8
Arm24
Facelower24
Jaw24
Larynx24
Neck24
Speech24
Tongue24

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.