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==Exosome==  
==Exosome==  
<StructureSection load='1stp' size='340' side='right' caption='Caption for this structure' scene=''>
<StructureSection load='2je6' size='340' side='right' caption='Exosome complex containing exonuclease 1 (green), exonuclease 2 (grey) and exosome complex RNA-binding protein 1 (wheat) complex with PEG400 (pdb code [[2je6]]).' scene=''>
This is the patiah
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). <ref>http://en.wikipedia.org/wiki/Exosome_complex#Structure</ref>
<ref>PMID:21638687</ref>


Some of the exosome components are:<br />
*'''MTR3''' and '''SKI6''' are part of RNase PH domain-containing subunits proposed to form a central channel which threads RNA for degradation.<br />
*'''MTR4''' interacts with Nop53 and together they have a role in the maturation of 5.8S rRNA<ref>PMID:28883156</ref>.<br />
*'''Rrp6''' is a nuclear-specific subunit promoting cell survival during heat stress<ref>PMID:32521279</ref>.<br />
*'''Rrp44''' is the catalytic subunit <ref>PMID:19060898</ref>.<br />
*'''Csl4''' is required for mRNA decay.<br />
*'''M-phase phosphoprotein 6''' is involved in the 3'-processing  of the 7S pre-RNA to the mature 5.8S rRNA.


__TOC__


== Function ==
== Function ==
<scene name='60/609824/2nn6/8'>Exosome</scene>
In order to see the structure of the protein complex, press <scene name='60/609824/2nn6/8'>Exosome</scene>.
 
===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 ==
== Disease ==
'''Autoimmunity'''
===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.
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'''
===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.
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.
== Relevance ==
 
===Neurological disorders===
Mutations in Exosome component 3 cause pontocerebellar hypoplasia and spinal motor neuron disease.


== Structural highlights ==
== Structural highlights ==
The core of the Exosom complex is made of a ring, which consisting of six RNases proteins (Rnase PH-like proteins).
The central machinery of exosome is shaped like a <scene name="/12/3456/Sample/1">barrel</scene>, with its RNA cleavage machinery sheltered in a groove inside.
This is a sample scene created with SAT to <scene name="/12/3456/Sample/1">color</scene> by Group, and another to make <scene name="/12/3456/Sample/2">a transparent representation</scene> of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.
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 <scene name="/12/3456/Sample/2">ribbon view</scene>.
 
==3D structures of exosome==
[[Exosome 3D structures]]


</StructureSection>
</StructureSection>
== References ==
== References ==
<references/>
<references/>
[[Category: Topic Page]]

Proteopedia Page Contributors and Editors (what is this?)Proteopedia Page Contributors and Editors (what is this?)

Nitzan Dubovski, Michal Harel