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''' | {{Large structure}} | ||
==Structural Basis of Mitochondrial Receptor Binding and Constriction by Dynamin-Related Protein 1== | |||
<StructureSection load='5wp9' size='340' side='right' caption='[[5wp9]], [[Resolution|resolution]] 4.22Å' scene=''> | |||
== Structural highlights == | |||
<table><tr><td colspan='2'>[[5wp9]] is a 16 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5WP9 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5WP9 FirstGlance]. <br> | |||
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GCP:PHOSPHOMETHYLPHOSPHONIC+ACID+GUANYLATE+ESTER'>GCP</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene></td></tr> | |||
<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Dynamin_GTPase Dynamin GTPase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.6.5.5 3.6.5.5] </span></td></tr> | |||
[[Category: | <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=5wp9 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5wp9 OCA], [http://pdbe.org/5wp9 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5wp9 RCSB], [http://www.ebi.ac.uk/pdbsum/5wp9 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5wp9 ProSAT]</span></td></tr> | ||
</table> | |||
{{Large structure}} | |||
== Disease == | |||
[[http://www.uniprot.org/uniprot/DNM1L_HUMAN DNM1L_HUMAN]] Note=May be associated with Alzheimer disease through beta-amyloid-induced increased S-nitrosylation of DNM1L, which triggers, directly or indirectly, excessive mitochondrial fission, synaptic loss and neuronal damage.<ref>PMID:19342591</ref> Defects in DNM1L are the cause of encephalopathy, lethal, due to defective mitochondrial and peroxisomal fission (EMPF) [MIM:[http://omim.org/entry/614388 614388]]. EMPF is a rare autosomal dominant systemic disorder resulting in lack of neurologic development and death in infancy. After birth, infants present in the first week of life with poor feeding and neurologic impairment, including hypotonia, little spontaneous movement, no tendon reflexes, no response to light stimulation, and poor visual fixation. Other features include mildly elevated plasma concentration of very-long-chain fatty acids, lactic acidosis, microcephaly, deep-set eyes, optic atrophy and hypoplasia, and an abnormal gyral pattern in both frontal lobes associated with dysmyelination.<ref>PMID:17460227</ref> <ref>PMID:19342591</ref> | |||
== Function == | |||
[[http://www.uniprot.org/uniprot/DNM1L_HUMAN DNM1L_HUMAN]] Functions in mitochondrial and peroxisomal division. Mediates membrane fission through oligomerization into ring-like structures which wrap around the scission site to constict and sever the mitochondrial membrane through a GTP hydrolysis-dependent mechanism. Required for normal brain development. Facilitates developmentally-regulated apoptosis during neural tube development. Required for a normal rate of cytochrome c release and caspase activation during apoptosis. Also required for mitochondrial fission during mitosis. Required for programmed necrosis execution. May be involved in vesicle transport.<ref>PMID:9570752</ref> <ref>PMID:9786947</ref> <ref>PMID:11514614</ref> <ref>PMID:12499366</ref> <ref>PMID:12618434</ref> <ref>PMID:15208300</ref> <ref>PMID:17015472</ref> <ref>PMID:17301055</ref> <ref>PMID:17553808</ref> <ref>PMID:17460227</ref> <ref>PMID:18695047</ref> <ref>PMID:18838687</ref> <ref>PMID:19638400</ref> <ref>PMID:19411255</ref> <ref>PMID:19342591</ref> <ref>PMID:20688057</ref> Isoform 1 and isoform 4 inhibit peroxisomal division when overexpressed.<ref>PMID:9570752</ref> <ref>PMID:9786947</ref> <ref>PMID:11514614</ref> <ref>PMID:12499366</ref> <ref>PMID:12618434</ref> <ref>PMID:15208300</ref> <ref>PMID:17015472</ref> <ref>PMID:17301055</ref> <ref>PMID:17553808</ref> <ref>PMID:17460227</ref> <ref>PMID:18695047</ref> <ref>PMID:18838687</ref> <ref>PMID:19638400</ref> <ref>PMID:19411255</ref> <ref>PMID:19342591</ref> <ref>PMID:20688057</ref> [[http://www.uniprot.org/uniprot/MID49_HUMAN MID49_HUMAN]] Mitochondrial outer membrane protein which regulates mitochondrial fission. Promotes the recruitment and association of the fission mediator dynamin-related protein 1 (DNM1L) to the mitochondrial surface independently of the mitochondrial fission FIS1 and MFF proteins. Regulates DNM1L GTPase activity.<ref>PMID:21508961</ref> <ref>PMID:23283981</ref> <ref>PMID:23530241</ref> <ref>PMID:23921378</ref> | |||
== References == | |||
<references/> | |||
__TOC__ | |||
</StructureSection> | |||
[[Category: Dynamin GTPase]] | |||
[[Category: Agard, D A]] | |||
[[Category: Frost, A]] | |||
[[Category: Kalia, R]] | |||
[[Category: Shaw, J M]] | |||
[[Category: Thomas, P V]] | |||
[[Category: Wang, R Y.R]] | |||
[[Category: Yusuf, A]] | |||
[[Category: Dynamin related-protein-1]] | |||
[[Category: Mid49]] | |||
[[Category: Mitochondrial division]] | |||
[[Category: Nucleotide]] | |||
[[Category: Protein fibril]] |