PROSITE documentation PDOC50957Josephin domain profile
The Josephin domain is an eukaryotic protein module of about 180 residues, which occurs in stand-alone form in Josephin-like proteins, and as an amino-terminal domain associated with two or three copies of the ubiquitin-interacting motif (UIM) (see <PDOC50330>) in ataxin 3-like proteins. Although it has originally been proposed that the Josephin domain could be an all-α helical domain distantly related to ENTH (see <PDOC50942>) and VHS (see <PDOC50179>) domains involved in membrane trafficking and regulatory adaptor function [1], it is now believed that it is a mainly α helical cysteine-protease domain predicted to be active against ubiquitin chains or related substrates [2,3].
The Josephin domain contains two conserved histidines and one cysteine that is required for the ubiquitin protease activity [2,3].
Some proteins known to contain a Josephin domain are listed below:
- Animal Machado-Joseph disease protein 1 (Ataxin 3). It interacts with key regulators (CBP, p300 and PCAF) of transcription and represses transcription.
- Plant Machado-Joseph disease-like protein (MJD1a-like) (Ataxin 3 homolog).
- Mammalian Josephin 1 and 2.
- Drosophila melanogaster Josephin-like protein.
- Arabidopsis thaliana Josephin-like protein.
The profile we developed spans the entire Josephin domain.
Last update:January 2004 / First entry.
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PROSITE method (with tools and information) covered by this documentation:
| 1 | Authors | Albrecht M. Hoffmann D. Evert B.O. Schmitt I. Wuellner U. Lengauer T. |
| Title | Structural modeling of ataxin-3 reveals distant homology to adaptins. | |
| Source | Proteins 50:355-370(2003). | |
| PubMed ID | 12486728 | |
| DOI | 10.1002/prot.10280 |
| 2 | Authors | Scheel H. Tomiuk S. Hofmann K. |
| Title | Elucidation of ataxin-3 and ataxin-7 function by integrative bioinformatics. | |
| Source | Hum. Mol. Genet. 12:2845-2852(2003). | |
| PubMed ID | 12944423 | |
| DOI | 10.1093/hmg/ddg297 |
| 3 | Authors | Burnett B. Li F. Pittman R.N. |
| Title | The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity. | |
| Source | Hum. Mol. Genet. 12:3195-3205(2003). | |
| PubMed ID | 14559776 | |
| DOI | 10.1093/hmg/ddg344 |
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