The human exosome: an autoantigenic complex of exoribonucleases in myositis and scleroderma
The anti-PM/Scl autoantibodies are known to characterize a subset of autoimmune patients with myositis, scleroderma (Scl), and the PM/Scl overlap syndrome. The major autoantigens that are recognized by anti-PM/Scl autoantibodies are designated PM/Scl-100 and PM/Scl-75. These autoantigens have been reported to associate into a large complex consisting of 11 to 16 proteins and to play a role in ribosome synthesis. Recently, it was discovered that the PM/Scl complex is the human counterpart of the yeast (Saccharomyces cerevisiae) exosome, which is an RNA-processing complex consisting of 11 3' → 5' exoribonucleases. To date, 10 human exosome components have been identified, although only some of these were studied in more detail. In this review, we discuss some recent advances in the characterization of the PM/Scl complex.
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Complete Metadata
| @type | dcat:Dataset |
|---|---|
| accessLevel | public |
| bureauCode |
[ "009:25" ] |
| contactPoint |
{ "fn": "NIH", "@type": "vcard:Contact", "hasEmail": "mailto:info@nih.gov" } |
| description | The anti-PM/Scl autoantibodies are known to characterize a subset of autoimmune patients with myositis, scleroderma (Scl), and the PM/Scl overlap syndrome. The major autoantigens that are recognized by anti-PM/Scl autoantibodies are designated PM/Scl-100 and PM/Scl-75. These autoantigens have been reported to associate into a large complex consisting of 11 to 16 proteins and to play a role in ribosome synthesis. Recently, it was discovered that the PM/Scl complex is the human counterpart of the yeast (Saccharomyces cerevisiae) exosome, which is an RNA-processing complex consisting of 11 3' → 5' exoribonucleases. To date, 10 human exosome components have been identified, although only some of these were studied in more detail. In this review, we discuss some recent advances in the characterization of the PM/Scl complex. |
| distribution |
[ { "@type": "dcat:Distribution", "title": "Official Government Data Source", "mediaType": "text/html", "description": "Visit the original government dataset for complete information, documentation, and data access.", "downloadURL": "https://www.ncbi.nlm.nih.gov/pmc/articles/PMC128886/" } ] |
| identifier | https://healthdata.gov/api/views/e4hh-a57m |
| issued | 2025-07-14 |
| keyword |
[ "autoimmune-diseases", "myositis", "nih", "pmscl-complex", "scleroderma" ] |
| landingPage | https://healthdata.gov/d/e4hh-a57m |
| modified | 2025-09-06 |
| programCode |
[ "009:033" ] |
| publisher |
{ "name": "National Institutes of Health", "@type": "org:Organization" } |
| theme |
[ "NIH" ] |
| title | The human exosome: an autoantigenic complex of exoribonucleases in myositis and scleroderma |
