The Exosome complex (or just- Exosome) is a multi-protein complex capable of degrading various types of RNA molecules. The Exosome complex is found in eukaryotic cell, and also in archaea, while in bacteria it is found as a simpler complex (but it has the same function). [1]
Function
In order to see the structure of the protein complex, press Exosome.
Enzymatic function
The exosome is primarily a 3'-5' exoribonuclease, meaning that it degrades RNA molecules from their 3' end. In eukaryotes it also have an endoribonucleolytic function, meaning it cleaves RNA at sites within the molecule.
Substrates
The exosome is involved in the degradation and processing of a wide variety of RNA species. Substrates of the exosome include messenger RNA, ribosomal RNA, and many species of small RNAs.
Disease
Autoimmunity
The exosome complex is the target of autoantibodies, which are known to be found in people that suffer from various autoimmune diseases (especially PM/Scl overlap syndrome). In the autoimune diseases, antibodies are mainly directed against two of the proteins of the complex, called PM/Scl-100 and PM/Scl-75.
Cancer treatment
The Exosome is found to be inhibited by a cancer chemotherapy drug, which called antimetabolite fluorouracil. This drug is one of the most successful drugs for treating solid tumors.
Neurological disorders
Mutations in Exosome component 3 cause pontocerebellar hypoplasia and spinal motor neuron disease.
Structural highlights
The central machinery of exosome is shaped like a barrel, with its RNA cleavage machinery sheltered in a groove inside.
The core of the Exosome complex is made of a ring, which is consisting of six RNases proteins (Rnase PH-like proteins), and other proteins are attached.
You can also view the complex in a ribbon view.
