CATH Classification

Domain Context

CATH Clusters

Superfamily 3.90.70.40
Functional Family Ataxin 3

Enzyme Information

3.4.19.12
Ubiquitinyl hydrolase 1.
based on mapping to UniProt P54252
Thiol-dependent hydrolysis of ester, thioester, amide, peptide and isopeptide bonds formed by the C-terminal Gly of ubiquitin (a 76-residue protein attached to proteins as an intracellular targeting signal).
-!- Links to polypeptides smaller than 60 residues are hydrolyzed more readily than those to larger polypeptides. -!- Isoforms exist with quantitatively different specificities among the best known being UCH-L1 and UCH-L3, major proteins of the brain of mammals. -!- Inhibited by ubiquitin aldehyde (in which Gly76 is replaced by aminoacetaldehyde). -!- Belongs to peptidase family C12.

UniProtKB Entries (1)

P54252
ATX3_HUMAN
Homo sapiens
Ataxin-3

PDB Structure

PDB 2AGA
External Links
Method SOLUTION NMR
Organism Escherichia
Primary Citation
Deubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain.
Mao, Y., Senic-Matuglia, F., Di Fiore, P.P., Polo, S., Hodsdon, M.E., De Camilli, P.
Proc.Natl.Acad.Sci.Usa
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