Your browser is unable to support new features implemented in HTML5 and CSS3 to render this site as intended. Your experience may suffer from functionality degradation but the site should remain usable. We strongly recommend the latest version of Google Chrome, OS X Safari or Mozilla Firefox. As Safari is bundled with OS X, if you are unable to upgrade to a newer version of OS X, we recommend using an open source browser. Dismiss message
IPR018978 is a Ribosome maturation protein SDO1/SBDS, central domain.
<p>This entry represents the central domain of proteins that are highly conserved in species ranging from archaea to vertebrates and plants [[cite:PUB00020303]]. This entry contains several Shwachman-Bodian-Diamond syndrome (SBDS) proteins from both mouse and humans. Shwachman-Diamond syndrome (OMIM 260400) is an autosomal recessive disorder with clinical features that include pancreatic exocrine insufficiency, haematological dysfunction and skeletal abnormalities. It is characterised by bone marrow failure and leukemia predisposition.</p> <p>Members of this entry play a role in RNA metabolism [[cite:PUB00020305], [cite:PUB00020304]]. In yeast, SBDS orthologue SDO1 is involved in the biogenesis of the 60S ribosomal subunit and translational activation of ribosomes. Together with the EF-2-like GTPase RIA1 (EfI1), it triggers the GTP-dependent release of TIF6 from 60S pre-ribosomes in the cytoplasm, thereby activating ribosomes for translation competence by allowing 80S ribosome assembly and facilitating TIF6 recycling to the nucleus, where it is required for 60S rRNA processing and nuclear export. This data links defective late 60S subunit maturation to an inherited bone marrow failure syndrome associated with leukemia predisposition [[cite:PUB00045126]].</p> <p>The SBDS protein is composed of three domains. The N-terminal (FYSH, [interpro:IPR019783]) domain is the most frequent target for disease mutations and contains a novel mixed α/β-fold, the central domain (represented in this entry) consists of a three-helical bundle and the C-terminal domain ([interpro:IPR046928]) has a ferredoxin-like fold [[cite:PUB00020305], [cite:PUB00076340]].</p>
This description is obtained from EB-eye REST.
GO predictions are based solely on the InterPro-to-GO mappings published by EMBL-EBI, which are in turn based on the mapping of predicted domains to the InterPro dataset. The InterPro-to-GO mapping was last updated on , while the GO metadata was last updated on .
| GO term | Namespace | Name | Definition | Relationships |
|---|---|---|---|---|
| Biological process | Ribosome biogenesis | A cellular process that results in the biosynthesis of constituent macromolecules, assembly, and arrangement of constituent parts of ribosome subunits; includes transport to the sites of protein synthesis. |
| Transcript | Name | Description | Predicted domains | Domain count |
|---|---|---|---|---|
| – | PREDICTED: ribosome maturation protein SBDS-like [Glycine max] gi|356552763|ref|XP_003544732.1| | 17 | ||
| – | Ribosome maturation protein SBDS; TAIR: AT1G43860.1 sequence-specific DNA binding transcription factor; Swiss-Prot: sp|P70122|SBDS_MOUSE Ribosome maturation protein SBDS; TrEMBL-Plants: tr|I3SB34|I3SB34_LOTJA Uncharacterized protein; Found in the gene: LotjaGi1g1v0167500 | 22 | ||
| – | Ribosome maturation protein SBDS; TAIR: AT1G43860.1 sequence-specific DNA binding transcription factor; Swiss-Prot: sp|P70122|SBDS_MOUSE Ribosome maturation protein SBDS; TrEMBL-Plants: tr|I3SB34|I3SB34_LOTJA Uncharacterized protein; Found in the gene: LotjaGi4g1v0306500 | 22 |
A list of co-occurring predicted domains within the L. japonicus gene space:
| Predicted domain | Source | Observations | Saturation (%) |
|---|---|---|---|
| mobidb-lite | MobiDBLite | 1 | 33.33 |