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{{STRUCTURE_4pqw| PDB=4pqw | SCENE= }} | |||
===Crystal Structure of Phospholipase C beta 3 in Complex with PDZ1 of NHERF1=== | |||
{{ABSTRACT_PUBMED_24642259}} | |||
==Disease== | |||
[[http://www.uniprot.org/uniprot/NHRF1_HUMAN NHRF1_HUMAN]] Defects in SLC9A3R1 are the cause of hypophosphatemic nephrolithiasis/osteoporosis type 2 (NPHLOP2) [MIM:[http://omim.org/entry/612287 612287]]. Hypophosphatemia results from idiopathic renal phosphate loss. It contributes to the pathogenesis of hypophosphatemic urolithiasis (formation of urinary calculi) as well to that of hypophosphatemic osteoporosis (bone demineralization).<ref>PMID:18784102</ref> <ref>PMID:22506049</ref> | |||
==Function== | |||
[[http://www.uniprot.org/uniprot/NHRF1_HUMAN NHRF1_HUMAN]] Scaffold protein that connects plasma membrane proteins with members of the ezrin/moesin/radixin family and thereby helps to link them to the actin cytoskeleton and to regulate their surface expression. Necessary for recycling of internalized ADRB2. Was first known to play a role in the regulation of the activity and subcellular location of SLC9A3. Necessary for cAMP-mediated phosphorylation and inhibition of SLC9A3. May enhance Wnt signaling. May participate in HTR4 targeting to microvilli (By similarity). Involved in the regulation of phosphate reabsorption in the renal proximal tubules.<ref>PMID:9430655</ref> <ref>PMID:9096337</ref> <ref>PMID:10499588</ref> <ref>PMID:18784102</ref> | |||
==About this Structure== | |||
[[4pqw]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4PQW OCA]. | |||
==Reference== | |||
<ref group="xtra">PMID:024642259</ref><references group="xtra"/><references/> | |||
[[Category: Brunzelle, J.]] | |||
[[Category: Guan, X.]] | |||
[[Category: Holcomb, J.]] | |||
[[Category: Hou, Y.]] | |||
[[Category: Jiang, Y.]] | |||
[[Category: Li, C.]] | |||
[[Category: Sirinupong, N.]] | |||
[[Category: Trescott, L.]] | |||
[[Category: Wang, S.]] | |||
[[Category: Yang, Z.]] | |||
[[Category: Nherf1]] | |||
[[Category: Pancreatic cancer]] | |||
[[Category: Plcb3]] | |||
[[Category: Protein binding]] | |||
[[Category: Scaffold protein]] | |||
Revision as of 12:13, 2 April 2014
Crystal Structure of Phospholipase C beta 3 in Complex with PDZ1 of NHERF1Crystal Structure of Phospholipase C beta 3 in Complex with PDZ1 of NHERF1
Template:ABSTRACT PUBMED 24642259
DiseaseDisease
[NHRF1_HUMAN] Defects in SLC9A3R1 are the cause of hypophosphatemic nephrolithiasis/osteoporosis type 2 (NPHLOP2) [MIM:612287]. Hypophosphatemia results from idiopathic renal phosphate loss. It contributes to the pathogenesis of hypophosphatemic urolithiasis (formation of urinary calculi) as well to that of hypophosphatemic osteoporosis (bone demineralization).[1] [2]
FunctionFunction
[NHRF1_HUMAN] Scaffold protein that connects plasma membrane proteins with members of the ezrin/moesin/radixin family and thereby helps to link them to the actin cytoskeleton and to regulate their surface expression. Necessary for recycling of internalized ADRB2. Was first known to play a role in the regulation of the activity and subcellular location of SLC9A3. Necessary for cAMP-mediated phosphorylation and inhibition of SLC9A3. May enhance Wnt signaling. May participate in HTR4 targeting to microvilli (By similarity). Involved in the regulation of phosphate reabsorption in the renal proximal tubules.[3] [4] [5] [6]
About this StructureAbout this Structure
4pqw is a 1 chain structure. Full crystallographic information is available from OCA.
ReferenceReference
- ↑ Jiang Y, Wang S, Holcomb J, Trescott L, Guan X, Hou Y, Brunzelle J, Sirinupong N, Li C, Yang Z. Crystallographic analysis of NHERF1-PLCbeta3 interaction provides structural basis for CXCR2 signaling in pancreatic cancer. Biochem Biophys Res Commun. 2014 Mar 15. pii: S0006-291X(14)00462-8. doi:, 10.1016/j.bbrc.2014.03.028. PMID:24642259 doi:http://dx.doi.org/10.1016/j.bbrc.2014.03.028
- ↑ Karim Z, Gerard B, Bakouh N, Alili R, Leroy C, Beck L, Silve C, Planelles G, Urena-Torres P, Grandchamp B, Friedlander G, Prie D. NHERF1 mutations and responsiveness of renal parathyroid hormone. N Engl J Med. 2008 Sep 11;359(11):1128-35. PMID:18784102 doi:359/11/1128
- ↑ Courbebaisse M, Leroy C, Bakouh N, Salaun C, Beck L, Grandchamp B, Planelles G, Hall RA, Friedlander G, Prie D. A new human NHERF1 mutation decreases renal phosphate transporter NPT2a expression by a PTH-independent mechanism. PLoS One. 2012;7(4):e34764. doi: 10.1371/journal.pone.0034764. Epub 2012 Apr 10. PMID:22506049 doi:10.1371/journal.pone.0034764
- ↑ Murthy A, Gonzalez-Agosti C, Cordero E, Pinney D, Candia C, Solomon F, Gusella J, Ramesh V. NHE-RF, a regulatory cofactor for Na(+)-H+ exchange, is a common interactor for merlin and ERM (MERM) proteins. J Biol Chem. 1998 Jan 16;273(3):1273-6. PMID:9430655
- ↑ Yun CH, Oh S, Zizak M, Steplock D, Tsao S, Tse CM, Weinman EJ, Donowitz M. cAMP-mediated inhibition of the epithelial brush border Na+/H+ exchanger, NHE3, requires an associated regulatory protein. Proc Natl Acad Sci U S A. 1997 Apr 1;94(7):3010-5. PMID:9096337
- ↑ Cao TT, Deacon HW, Reczek D, Bretscher A, von Zastrow M. A kinase-regulated PDZ-domain interaction controls endocytic sorting of the beta2-adrenergic receptor. Nature. 1999 Sep 16;401(6750):286-90. PMID:10499588 doi:10.1038/45816
- ↑ Karim Z, Gerard B, Bakouh N, Alili R, Leroy C, Beck L, Silve C, Planelles G, Urena-Torres P, Grandchamp B, Friedlander G, Prie D. NHERF1 mutations and responsiveness of renal parathyroid hormone. N Engl J Med. 2008 Sep 11;359(11):1128-35. PMID:18784102 doi:359/11/1128