The UMD-THAP1 mutations database
Record ID: 36

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.407A>Gp.Asn136SerHomozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AACAsnAGCSerA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
 HCF-1 binding Yes, non coding strandNo

Mutation impact


At the mRNA levelOn restriction map
Potential acceptor splice site createdNew restriction site(s): Alu I
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
10.13 (non pathogenous)71 (Probably pathogenous)

Patient and sample data


Sample IDPatient IDFamily IDPatient statusGenderTransmissionGeographic origin
UKD02LON F0008 I0001-Family CProbandMaleNo family historyMAURITANIA

Phenotypic groupDisease
NASegmental dystonia

Clinical data


SymptomSeverityAge
Age at last examination69
Age of onsethand57
Armbilateral69
Neck69

Reference


Reference IDPubMed IDReference
820211909
Houlden H, Schneider SA, Paudel R, Melchers A, Schwingenschuh P, Edwards M, Hardy J, Bhatia KP. THAP1 mutations (DYT6) are an additional cause of early-onset dystonia. Neurology. 2010 Mar 9;74(10):846-50.