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==CRYSTAL STRUCTURE OF THE ACTIN-BINDING DOMAIN OF HUMAN ALPHA-ACTININ-4== | |||
<StructureSection load='6oa6' size='340' side='right'caption='[[6oa6]], [[Resolution|resolution]] 1.37Å' scene=''> | |||
== Structural highlights == | |||
<table><tr><td colspan='2'>[[6oa6]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6OA6 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6OA6 FirstGlance]. <br> | |||
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene></td></tr> | |||
[[Category: | <tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[6o31|6o31]], [[2r0o|2r0o]]</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=6oa6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6oa6 OCA], [http://pdbe.org/6oa6 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6oa6 RCSB], [http://www.ebi.ac.uk/pdbsum/6oa6 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6oa6 ProSAT]</span></td></tr> | |||
</table> | |||
== Disease == | |||
[[http://www.uniprot.org/uniprot/ACTN4_HUMAN ACTN4_HUMAN]] Defects in ACTN4 are the cause of focal segmental glomerulosclerosis type 1 (FSGS1) [MIM:[http://omim.org/entry/603278 603278]]. A renal pathology defined by the presence of segmental sclerosis in glomeruli and resulting in proteinuria, reduced glomerular filtration rate and edema. Renal insufficiency often progresses to end-stage renal disease, a highly morbid state requiring either dialysis therapy or kidney transplantation.<ref>PMID:10700177</ref> | |||
== Function == | |||
[[http://www.uniprot.org/uniprot/ACTN4_HUMAN ACTN4_HUMAN]] F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein. Probably involved in vesicular trafficking via its association with the CART complex. The CART complex is necessary for efficient transferrin receptor recycling but not for EGFR degradation. | |||
== References == | |||
<references/> | |||
__TOC__ | |||
</StructureSection> | |||
[[Category: Large Structures]] | |||
[[Category: Birrane, G]] | |||
[[Category: Feng, D]] | [[Category: Feng, D]] | ||
[[Category: | [[Category: Actin- binding protein]] | ||
[[Category: Actin-crosslinking]] | |||
[[Category: Calponin homology domain]] | |||
[[Category: Ch domain]] | |||
[[Category: Disease mutation]] | |||
[[Category: Glomeruloscleros spectrin family]] | |||
[[Category: Nucleus]] | |||
[[Category: Phosphorylation]] | |||
[[Category: Structural protein]] | |||
Revision as of 12:42, 18 March 2020
CRYSTAL STRUCTURE OF THE ACTIN-BINDING DOMAIN OF HUMAN ALPHA-ACTININ-4CRYSTAL STRUCTURE OF THE ACTIN-BINDING DOMAIN OF HUMAN ALPHA-ACTININ-4
Structural highlights
Disease[ACTN4_HUMAN] Defects in ACTN4 are the cause of focal segmental glomerulosclerosis type 1 (FSGS1) [MIM:603278]. A renal pathology defined by the presence of segmental sclerosis in glomeruli and resulting in proteinuria, reduced glomerular filtration rate and edema. Renal insufficiency often progresses to end-stage renal disease, a highly morbid state requiring either dialysis therapy or kidney transplantation.[1] Function[ACTN4_HUMAN] F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein. Probably involved in vesicular trafficking via its association with the CART complex. The CART complex is necessary for efficient transferrin receptor recycling but not for EGFR degradation. References
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