The UMD-THAP1 mutations database
Record ID: 175

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.151A>Gp.Ser51GlyHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AGCSerGGCGlyA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
L3-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)
0.790.06 (non pathogenous)53 (Probable polymorphism)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
NET01AMS F0004 I0001ProbandFemaleNo family historyNETHERLAND

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination35
Age of onsetlarynx14
Larynx14
Tongue35

Reference


Reference IDPubMed IDReference
29 21538522
Groen JL, Yildirim E, Ritz K, Baas F, van Hilten JJ, van der Meulen FW, Langeveld TP, Tijssen MA. THAP1 mutations are infrequent in spasmodic dysphonia. Mov Disord. 2011 Aug 15;26(10):1952-4.