4bej

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Current revision (11:49, 20 December 2023) (edit) (undo)
 
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<StructureSection load='4bej' size='340' side='right'caption='[[4bej]], [[Resolution|resolution]] 3.48&Aring;' scene=''>
<StructureSection load='4bej' size='340' side='right'caption='[[4bej]], [[Resolution|resolution]] 3.48&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[4bej]] is a 4 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4BEJ OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4BEJ FirstGlance]. <br>
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<table><tr><td colspan='2'>[[4bej]] is a 4 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=4BEJ OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4BEJ FirstGlance]. <br>
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</td></tr><tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[https://en.wikipedia.org/wiki/Dynamin_GTPase Dynamin GTPase], with EC number [https://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.6.5.5 3.6.5.5] </span></td></tr>
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 3.483&#8491;</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=4bej FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4bej OCA], [https://pdbe.org/4bej PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4bej RCSB], [https://www.ebi.ac.uk/pdbsum/4bej PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4bej ProSAT]</span></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=4bej FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4bej OCA], [https://pdbe.org/4bej PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4bej RCSB], [https://www.ebi.ac.uk/pdbsum/4bej PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4bej ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
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[[https://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:[https://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>
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[https://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:[https://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 ==
== Function ==
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[[https://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>
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[https://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>
<div style="background-color:#fffaf0;">
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
== Publication Abstract from PubMed ==
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__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Dynamin GTPase]]
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[[Category: Homo sapiens]]
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[[Category: Human]]
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[[Category: Large Structures]]
[[Category: Large Structures]]
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[[Category: Daumke, O]]
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[[Category: Daumke O]]
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[[Category: Faelber, K]]
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[[Category: Faelber K]]
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[[Category: Froehlich, C]]
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[[Category: Froehlich C]]
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[[Category: Schwefel, D]]
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[[Category: Schwefel D]]
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[[Category: Apoptosis]]
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[[Category: G protein]]
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[[Category: Hydrolase]]
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[[Category: Membrane remodeling]]
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[[Category: Mitochondrial fission]]
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Current revision

Nucleotide-free Dynamin 1-like protein (DNM1L, DRP1, DLP1)

PDB ID 4bej

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Proteopedia Page Contributors and Editors (what is this?)

OCA

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