Family C86


Summary Holotypes Alignment Tree Genomes Structure Literature H-seq M-seq

Summary for family C86

Family type peptidaseC86.001 - ataxin-3 (Homo sapiens), MEROPS Accession MER0099998 (peptidase unit: 1-180)
Content of familyFamily C86 contains deubiquitinylating peptidases.
History Identifier created: MEROPS 8.00A (14 February 2008)
Ataxin-3, the protein mutated in spinocerebellar ataxia type 3 (Machado Joseph disease), has been found to have deubiquitinylating activity.
Catalytic typeCysteine
Active site residuesC14 H119 
Active siteThe sequence of ataxin-3 clearly places family C86 in clan CA, with some resemblance to families C12 and C19. The putative catalytic Cys14 (numbering as in our Alignment) was found to be essential for deubiquitinylating activity (Rodrigues et al., 2007).
Activities and specificitiesHuman ataxin-3 was proposed by Scheel et al. (2003) to have deubiquitinylating activity, and work with model organisms has confirmed this activity (Rodrigues et al., 2007).
InhibitorsUbiquitin aldehyde (Burnett et al., 2003), E64 and CA074 (Nicastro et al., 2005).
Molecular structureThe NMR solution structure of the Josephin domain of ataxin-3, containing the active site, was determined by Nicastro et al. (2005); it shows the characteristics of a cysteine peptidase in clan CA.
Distribution of family Bacteria -  
Archaea -  
Protozoa details  
Fungi details  
Plants details  
Animals details  
Viruses -  
Biological functionsA knockout of ataxin-3 in Caenorhabditis elegans showed that the deficient individuals were viable and displayed no gross phenotype. Large-scale microarray analysis revealed deregulation of genes involved in the ubiquitin-proteasome pathway, structure/motility, and signal transduction (Rodrigues et al., 2007).
Statistics for family C86Sequences:1271
Identifiers with PDB entries:2
Downloadable files Sequence library (FastA format)
Sequence alignment (FastA format)
Phylogenetic tree (Newick format)
Peptidases and Homologues MEROPS ID Structure
ataxin-3-like peptidaseC86.002Yes
ataxin-3 homologue (Caenorhabditis sp.)C86.003-
JosD1 peptidaseC86.004-
JosD2 peptidaseC86.005-
At3g54130 (Arabidopsis thaliana)C86.A01-
At2g29640 (Arabidopsis thaliana)C86.A02-
Y71H2AR.3 g.p. (Caenorhabditis elegans)C86.A03-
PF11_0125 g.p. (Plasmodium falciparum)C86.A04-
PFL1295w g.p. (Plasmodium falciparum)C86.A05-
family C86 non-peptidase homologuesnon-peptidase homologue-
family C86 unassigned peptidasesunassigned-