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5zrv

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Revision as of 22:54, 10 April 2020

Structure of human mitochondrial trifunctional protein, octamer

5zrv, resolution 7.70Å

Structural highlights

5zrv is a 8 chain structure with sequence from Human. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Gene:	HADHA, HADH (HUMAN), HADHB, MSTP029 (HUMAN)
Activity:	Acetyl-CoA C-acyltransferase, with EC number 2.3.1.16
Experimental data:	Check to display Experimental Data
Resources:	FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

[ECHA_HUMAN] Mitochondrial trifunctional protein deficiency;Acute fatty liver of pregnancy;Long chain 3-hydroxyacyl-CoA dehydrogenase deficiency. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. [ECHB_HUMAN] Mitochondrial trifunctional protein deficiency. The disease is caused by mutations affecting the gene represented in this entry.

Function

[ECHA_HUMAN] Bifunctional subunit.

Publication Abstract from PubMed

The mitochondrial trifunctional protein (TFP) catalyzes three reactions in the fatty acid beta-oxidation process. Mutations in the two TFP subunits cause mitochondrial trifunctional protein deficiency and acute fatty liver of pregnancy that can lead to death. Here we report a 4.2-A cryo-electron microscopy alpha2beta2 tetrameric structure of the human TFP. The tetramer has a V-shaped architecture that displays a distinct assembly compared with the bacterial TFPs. A concave surface of the TFP tetramer interacts with the detergent molecules in the structure, suggesting that this region is involved in associating with the membrane. Deletion of a helical hairpin in TFPbeta decreases its binding to the liposomes in vitro and reduces its membrane targeting in cells. Our results provide the structural basis for TFP function and have important implications for fatty acid oxidation related diseases.

Cryo-EM structure of human mitochondrial trifunctional protein.,Liang K, Li N, Wang X, Dai J, Liu P, Wang C, Chen XW, Gao N, Xiao J Proc Natl Acad Sci U S A. 2018 Jun 18. pii: 1801252115. doi:, 10.1073/pnas.1801252115. PMID:29915090^[1]

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

References

↑ Liang K, Li N, Wang X, Dai J, Liu P, Wang C, Chen XW, Gao N, Xiao J. Cryo-EM structure of human mitochondrial trifunctional protein. Proc Natl Acad Sci U S A. 2018 Jun 18. pii: 1801252115. doi:, 10.1073/pnas.1801252115. PMID:29915090 doi:http://dx.doi.org/10.1073/pnas.1801252115

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

Proteopedia Page Contributors and Editors (what is this?)

OCA

Retrieved from "http://52.214.119.220/wiki/index.php/5zrv"

@@ Line 3: / Line 3: @@
 <SX load='5zrv' size='340' side='right' viewer='molstar' caption='[[5zrv]], [[Resolution|resolution]] 7.70&Aring;' scene=''>
 == Structural highlights ==
-<table><tr><td colspan='2'>[[5zrv]] is a 8 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5ZRV OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5ZRV FirstGlance]. <br>
+<table><tr><td colspan='2'>[[5zrv]] is a 8 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5ZRV OCA]. For a <b>guided tour on the structure components</b> use [http://proteopedia.org/fgij/fg.htm?mol=5ZRV FirstGlance]. <br>
 </td></tr><tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">HADHA, HADH ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN]), HADHB, MSTP029 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
 <tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Acetyl-CoA_C-acyltransferase Acetyl-CoA C-acyltransferase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.3.1.16 2.3.1.16] </span></td></tr>
-<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5zrv FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5zrv OCA], [http://pdbe.org/5zrv PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5zrv RCSB], [http://www.ebi.ac.uk/pdbsum/5zrv PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5zrv ProSAT]</span></td></tr>
+<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://proteopedia.org/fgij/fg.htm?mol=5zrv FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5zrv OCA], [http://pdbe.org/5zrv PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5zrv RCSB], [http://www.ebi.ac.uk/pdbsum/5zrv PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5zrv ProSAT]</span></td></tr>
 </table>
 == Disease ==

5zrv

From Proteopedia

Revision as of 22:54, 10 April 2020

Structure of human mitochondrial trifunctional protein, octamer

Structural highlights

Disease

Function

Publication Abstract from PubMed

References

Contents

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