3so6

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{{STRUCTURE_3so6| PDB=3so6 | SCENE= }}
 
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===Crystal structure of the LDL receptor tail in complex with autosomal recessive hypercholesterolemia PTB domain===
 
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{{ABSTRACT_PUBMED_22509010}}
 
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==Disease==
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==Crystal structure of the LDL receptor tail in complex with autosomal recessive hypercholesterolemia PTB domain==
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[[http://www.uniprot.org/uniprot/LDLR_HUMAN LDLR_HUMAN]] Defects in LDLR are the cause of familial hypercholesterolemia (FH) [MIM:[http://omim.org/entry/143890 143890]]; a common autosomal semi-dominant disease that affects about 1 in 500 individuals. The receptor defect impairs the catabolism of LDL, and the resultant elevation in plasma LDL-cholesterol promotes deposition of cholesterol in the skin (xanthelasma), tendons (xanthomas), and coronary arteries (atherosclerosis).<ref>PMID:3263645</ref><ref>PMID:2569482</ref><ref>PMID:3955657</ref><ref>PMID:8347689</ref><ref>PMID:2318961</ref><ref>PMID:1446662</ref><ref>PMID:1867200</ref><ref>PMID:8462973</ref><ref>PMID:8168830</ref><ref>PMID:2726768</ref><ref>PMID:1464748</ref><ref>PMID:7573037</ref><ref>PMID:7583548</ref><ref>PMID:7550239</ref><ref>PMID:7635461</ref><ref>PMID:7635482</ref><ref>PMID:7649546</ref><ref>PMID:7649549</ref><ref>PMID:8740918</ref><ref>PMID:8664907</ref><ref>PMID:9026534</ref><ref>PMID:9254862</ref><ref>PMID:9143924</ref><ref>PMID:9259195</ref><ref>PMID:9104431</ref><ref>PMID:9654205</ref><ref>PMID:9452094</ref><ref>PMID:9452095</ref><ref>PMID:9452118</ref><ref>PMID:10206683</ref><ref>PMID:10660340</ref>[:]<ref>PMID:9852677</ref><ref>PMID:9678702</ref><ref>PMID:10422803</ref><ref>PMID:10090484</ref><ref>PMID:10447263</ref><ref>PMID:10978268</ref><ref>PMID:10980548</ref><ref>PMID:10882754</ref><ref>PMID:11298688</ref><ref>PMID:17142622</ref><ref>PMID:19319977</ref><ref>PMID:22160468</ref>
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<StructureSection load='3so6' size='340' side='right'caption='[[3so6]], [[Resolution|resolution]] 1.37&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[3so6]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] and [https://en.wikipedia.org/wiki/Rattus_norvegicus Rattus norvegicus]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3SO6 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3SO6 FirstGlance]. <br>
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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]] 1.37&#8491;</td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=3so6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3so6 OCA], [https://pdbe.org/3so6 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3so6 RCSB], [https://www.ebi.ac.uk/pdbsum/3so6 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3so6 ProSAT]</span></td></tr>
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</table>
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== Function ==
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[https://www.uniprot.org/uniprot/ARH_RAT ARH_RAT] Adapter protein (clathrin-associated sorting protein (CLASP)) required for efficient endocytosis of the LDL receptor (LDLR) in polarized cells such as hepatocytes and lymphocytes, but not in non-polarized cells (fibroblasts). May be required for LDL binding and internalization but not for receptor clustering in coated pits. May facilitate the endocytosis of LDLR and LDLR-LDL complexes from coated pits by stabilizing the interaction between the receptor and the structural components of the pits. May also be involved in the internalization of other LDLR family members. Binds to phosphoinositides, which regulate clathrin bud assembly at the cell surface (By similarity). Required for trafficking of LRP2 to the endocytic recycling compartment which is necessary for LRP2 proteolysis, releasing a tail fragment which translocates to the nucleus and mediates transcriptional repression (PubMed:23836931).[UniProtKB:Q8C142]<ref>PMID:23836931</ref>
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==Function==
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==See Also==
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[[http://www.uniprot.org/uniprot/LDLR_HUMAN LDLR_HUMAN]] Binds LDL, the major cholesterol-carrying lipoprotein of plasma, and transports it into cells by endocytosis. In order to be internalized, the receptor-ligand complexes must first cluster into clathrin-coated pits. In case of HIV-1 infection, functions as a receptor for extracellular Tat in neurons, mediating its internalization in uninfected cells.
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*[[LDL receptor|LDL receptor]]
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== References ==
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==About this Structure==
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<references/>
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[[3so6]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Rattus_norvegicus Rattus norvegicus]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3SO6 OCA].
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__TOC__
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</StructureSection>
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==Reference==
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[[Category: Homo sapiens]]
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<ref group="xtra">PMID:022509010</ref><references group="xtra"/><references/>
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[[Category: Large Structures]]
[[Category: Rattus norvegicus]]
[[Category: Rattus norvegicus]]
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[[Category: Dvir, H.]]
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[[Category: Dvir H]]
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[[Category: Zajonc, D M.]]
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[[Category: Zajonc DM]]
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[[Category: Arh]]
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[[Category: Autosomal recessive hypercholesterolemia]]
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[[Category: Cholesterol]]
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[[Category: Endocytic adaptor]]
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[[Category: Ldl]]
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[[Category: Ldlr]]
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[[Category: Protein binding-protein transport complex]]
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[[Category: Ptb]]
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[[Category: Receptor]]
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Current revision

Crystal structure of the LDL receptor tail in complex with autosomal recessive hypercholesterolemia PTB domain

PDB ID 3so6

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