2rsm

From Proteopedia

(Difference between revisions)

Current revision

Solution structure and siRNA-mediated knockdown analysis of the mitochondrial disease-related protein C12orf65 (ICT2)

Structural highlights

2rsm is a 1 chain structure with sequence from Mus musculus. Full experimental information is available from OCA. For a guided tour on the structure components use FirstGlance.
Resources:	FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Function

MTRFR_MOUSE Part of a mitoribosome-associated quality control pathway that prevents aberrant translation by responding to interruptions during elongation. As heterodimer with MTRES1, ejects the unfinished nascent chain and peptidyl transfer RNA (tRNA), respectively, from stalled ribosomes. Recruitment of mitoribosome biogenesis factors to these quality control intermediates suggests additional roles for MTRES1 and MTRF during mitoribosome rescue.[UniProtKB:Q9H3J6]

Publication Abstract from PubMed

Loss of function of the c12orf65 gene causes a mitochondrial translation defect, leading to encephalomyopathy. The C12orf65 protein is thought to play a role similar to that of ICT1 in rescuing stalled mitoribosomes during translation. Both proteins belong to a family of class I peptide release factors (RFs), all characterized by the presence of a GGQ motif. Here, we determined the solution structure of the GGQ-containing domain (GGQ domain) of C12orf65 from mouse by NMR spectroscopy, and examined the effect of siRNA-mediated knockdown of C12orf65 on mitochondria in HeLa cells using flow cytometry. The GGQ domain, comprising residues 60-124 of the 184-residue full-length protein, forms a structure with a 3(10) -beta1-beta2-beta3-alpha1 topology that resembles the GGQ domain structure of RF more closely than that of ICT1. Thus, the GGQ domain structures of this protein family can be divided into two types, depending on the region linking beta2 and beta3; the C12orf65/RF type having a 6-residue pi-HB turn and the ICT1 type having an alpha-helix. Knockdown of C12orf65 resulted in increased ROS production and apoptosis, leading to inhibition of cell proliferation. Substantial changes in mitochondrial membrane potential and mass in the C12orf65-knockdown cells were observed compared to the control cells. These results indicate that the function of C12orf65 is essential for cell vitality and mitochondrial function. Whereas similar effects were observed in ICT1-downregulated cells, there were significant differences in the range and pattern of the effects between C12orf65- and ICT1-knockdown cells, suggesting different roles of C12orf65 and ICT1 in rescuing stalled mitoribosomes. Proteins 2012. (c) 2012 Wiley Periodicals, Inc.

Solution structure and siRNA-mediated knockdown analysis of the mitochondrial disease-related protein C12orf65.,Kogure H, Hikawa Y, Hagihara M, Tochio N, Koshiba S, Inoue Y, Guntert P, Kigawa T, Yokoyama S, Nameki N Proteins. 2012 Jul 21. doi: 10.1002/prot.24152. PMID:22821833^[1]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

References

↑ Kogure H, Hikawa Y, Hagihara M, Tochio N, Koshiba S, Inoue Y, Guntert P, Kigawa T, Yokoyama S, Nameki N. Solution structure and siRNA-mediated knockdown analysis of the mitochondrial disease-related protein C12orf65. Proteins. 2012 Jul 21. doi: 10.1002/prot.24152. PMID:22821833 doi:10.1002/prot.24152

1 Structural highlights
2 Function
3 Publication Abstract from PubMed
4 References

Proteopedia Page Contributors and Editors (what is this?)

OCA

Retrieved from "http://52.214.119.220/wiki/index.php/2rsm"

@@ Line 1: / Line 1: @@
 ==Solution structure and siRNA-mediated knockdown analysis of the mitochondrial disease-related protein C12orf65 (ICT2)==
-<StructureSection load='2rsm' size='340' side='right'caption='[[2rsm]], [[NMR_Ensembles_of_Models | 20 NMR models]]' scene=''>
+<StructureSection load='2rsm' size='340' side='right'caption='[[2rsm]]' scene=''>
 == Structural highlights ==
-<table><tr><td colspan='2'>[[2rsm]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Lk3_transgenic_mice Lk3 transgenic mice]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2RSM OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2RSM FirstGlance]. <br>
+<table><tr><td colspan='2'>[[2rsm]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Mus_musculus Mus musculus]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2RSM OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2RSM FirstGlance]. <br>
 </td></tr><tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2rsm FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2rsm OCA], [https://pdbe.org/2rsm PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2rsm RCSB], [https://www.ebi.ac.uk/pdbsum/2rsm PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2rsm ProSAT]</span></td></tr>
 </table>
 == Function ==
-[[https://www.uniprot.org/uniprot/CL065_MOUSE CL065_MOUSE]] May act as a codon-independent translation release factor that has lost all stop codon specificity and directs the termination of translation in mitochondrion (By similarity).
+[https://www.uniprot.org/uniprot/MTRFR_MOUSE MTRFR_MOUSE] Part of a mitoribosome-associated quality control pathway that prevents aberrant translation by responding to interruptions during elongation. As heterodimer with MTRES1, ejects the unfinished nascent chain and peptidyl transfer RNA (tRNA), respectively, from stalled ribosomes. Recruitment of mitoribosome biogenesis factors to these quality control intermediates suggests additional roles for MTRES1 and MTRF during mitoribosome rescue.[UniProtKB:Q9H3J6]
 <div style="background-color:#fffaf0;">
 == Publication Abstract from PubMed ==
@@ Line 22: / Line 22: @@
 </StructureSection>
 [[Category: Large Structures]]
-[[Category: Lk3 transgenic mice]]
+[[Category: Mus musculus]]
-[[Category: Enomoto, M]]
+[[Category: Enomoto M]]
-[[Category: Guntert, P]]
+[[Category: Guntert P]]
-[[Category: Kigawa, T]]
+[[Category: Kigawa T]]
-[[Category: Koshiba, S]]
+[[Category: Koshiba S]]
-[[Category: Nameki, N]]
+[[Category: Nameki N]]
-[[Category: Tochio, N]]
+[[Category: Tochio N]]
-[[Category: Tomizawa, T]]
+[[Category: Tomizawa T]]
-[[Category: Yokoyama, S]]
+[[Category: Yokoyama S]]
-[[Category: Ggq domain]]
-[[Category: Release factor]]
-[[Category: Translation]]

2rsm

From Proteopedia

Current revision

Solution structure and siRNA-mediated knockdown analysis of the mitochondrial disease-related protein C12orf65 (ICT2)

Structural highlights

Function

Publication Abstract from PubMed

References

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Proteopedia Page Contributors and Editors (what is this?)

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