The UMD-THAP1 mutations database
Record ID: 19

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.508T>Cp.Cys170ArgHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGCCysCGCArgT->CTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
Coiled-Coil dom. NoNo

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)
0.860.00 (pathogenous)94 (Pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
ITA01ROM F0001 I0001case 1-ProbandMaleNo family historyGREECE

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination57
Age of onsethand10
Arm10
NeckSevere16

Reference


Reference IDPubMed IDReference
619908325
Bonetti M, Barzaghi C, Brancati F, Ferraris A, Bellacchio E, Giovanetti A, Ialongo T, Zorzi G, Piano C, Petracca M, Albanese A, Nardocci N, Dallapiccola B, Bentivoglio AR, Garavaglia B, Valente EM. Mutation screening of the DYT6/THAP1 gene in Italy. Mov Disord. 2009 Dec 15;24(16):2424-7.