7s7g: Difference between revisions
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==Crystal Structure Analysis of Human VLCAD== | |||
<StructureSection load='7s7g' size='340' side='right'caption='[[7s7g]], [[Resolution|resolution]] 1.34Å' scene=''> | |||
== Structural highlights == | |||
<table><tr><td colspan='2'>[[7s7g]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=7S7G OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=7S7G FirstGlance]. <br> | |||
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene></td></tr> | |||
[[Category: | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=7s7g FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=7s7g OCA], [https://pdbe.org/7s7g PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=7s7g RCSB], [https://www.ebi.ac.uk/pdbsum/7s7g PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=7s7g ProSAT]</span></td></tr> | ||
</table> | |||
== Disease == | |||
[[https://www.uniprot.org/uniprot/ACADV_HUMAN ACADV_HUMAN]] Defects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD) [MIM:[https://omim.org/entry/201475 201475]]. ACADVLD is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting.<ref>PMID:8554073</ref> <ref>PMID:9546340</ref> <ref>PMID:10077518</ref> | |||
== Function == | |||
[[https://www.uniprot.org/uniprot/ACADV_HUMAN ACADV_HUMAN]] Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.<ref>PMID:18227065</ref> | |||
== References == | |||
<references/> | |||
__TOC__ | |||
</StructureSection> | |||
[[Category: Homo sapiens]] | |||
[[Category: Large Structures]] | |||
[[Category: Dhe-Paganon S]] | |||
[[Category: Seo H-S]] | |||
Revision as of 06:14, 28 September 2022
Crystal Structure Analysis of Human VLCAD
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