4bsu

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m (Protected "4bsu" [edit=sysop:move=sysop])
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'''Unreleased structure'''
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{{STRUCTURE_4bsu| PDB=4bsu | SCENE= }}
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===Structure of the ectodomain of LGR5 in complex with R-spondin-1 (Fu1Fu2) in C2 crystal form===
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The entry 4bsu is ON HOLD
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==Disease==
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[[http://www.uniprot.org/uniprot/RSPO1_HUMAN RSPO1_HUMAN]] Palmoplantar keratoderma - XX sex reversal - predisposition to squamous cell carcinoma. Keratoderma, palmoplantar, with squamous cell carcinoma of skin and sex reversal (PKKSCC) [MIM:[http://omim.org/entry/610644 610644]]: A recessive syndrome characterized by XX (female to male) SRY-independent sex reversal, palmoplantar hyperkeratosis and predisposition to squamous cell carcinoma of the skin. Note=The disease is caused by mutations affecting the gene represented in this entry.
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Authors: Peng, W.C., de Lau, W., Forneris, F., Granneman, J.C.M., Huch, M., Clevers, H., Gros, P.
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==Function==
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[[http://www.uniprot.org/uniprot/LGR5_HUMAN LGR5_HUMAN]] Orphan receptor. Stem cell marker of the intestinal epithelium and the hair follicle. Target gene of Wnt signaling. [[http://www.uniprot.org/uniprot/RSPO1_HUMAN RSPO1_HUMAN]] Activator of the beta-catenin signaling cascade, leading to TCF-dependent gene activation. Acts both in the canonical Wnt/beta-catenin-dependent pathway and in non-canonical Wnt signaling pathway, probably by acting as an inhibitor of ZNRF3, an important regulator of the Wnt signaling pathway. Acts as a ligand for frizzled FZD8 and LRP6. May negatively regulate the TGF-beta pathway. Has a essential roles in ovary determination.<ref>PMID:16109882</ref> <ref>PMID:22575959</ref>
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Description: Structure of the ectodomain of LGR5 in complex with R-spondin-1 ( Fu1Fu2) in C2 crystal form
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==About this Structure==
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[[4bsu]] is a 8 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4BSU OCA].
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==Reference==
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<references group="xtra"/><references/>
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[[Category: Homo sapiens]]
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[[Category: Clevers, H.]]
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[[Category: Forneris, F.]]
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[[Category: Granneman, J C.M.]]
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[[Category: Gros, P.]]
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[[Category: Huch, M.]]
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[[Category: Lau, W de.]]
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[[Category: Peng, W C.]]
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[[Category: Adult stem cell]]
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[[Category: Congenital anonychia]]
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[[Category: Furin domain]]
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[[Category: Leucine-rich repeat]]
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[[Category: Leucine-rich repeat g-protein coupled receptor]]
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[[Category: Signaling protein]]
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[[Category: Signalling protein]]
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[[Category: Wnt signaling]]

Revision as of 10:23, 30 June 2013

Template:STRUCTURE 4bsu

Contents

Structure of the ectodomain of LGR5 in complex with R-spondin-1 (Fu1Fu2) in C2 crystal form

Disease

[RSPO1_HUMAN] Palmoplantar keratoderma - XX sex reversal - predisposition to squamous cell carcinoma. Keratoderma, palmoplantar, with squamous cell carcinoma of skin and sex reversal (PKKSCC) [MIM:610644]: A recessive syndrome characterized by XX (female to male) SRY-independent sex reversal, palmoplantar hyperkeratosis and predisposition to squamous cell carcinoma of the skin. Note=The disease is caused by mutations affecting the gene represented in this entry.

Function

[LGR5_HUMAN] Orphan receptor. Stem cell marker of the intestinal epithelium and the hair follicle. Target gene of Wnt signaling. [RSPO1_HUMAN] Activator of the beta-catenin signaling cascade, leading to TCF-dependent gene activation. Acts both in the canonical Wnt/beta-catenin-dependent pathway and in non-canonical Wnt signaling pathway, probably by acting as an inhibitor of ZNRF3, an important regulator of the Wnt signaling pathway. Acts as a ligand for frizzled FZD8 and LRP6. May negatively regulate the TGF-beta pathway. Has a essential roles in ovary determination.[1] [2]

About this Structure

4bsu is a 8 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  1. ↑ Kim KA, Kakitani M, Zhao J, Oshima T, Tang T, Binnerts M, Liu Y, Boyle B, Park E, Emtage P, Funk WD, Tomizuka K. Mitogenic influence of human R-spondin1 on the intestinal epithelium. Science. 2005 Aug 19;309(5738):1256-9. PMID:16109882 doi:309/5738/1256
  2. ↑ Hao HX, Xie Y, Zhang Y, Charlat O, Oster E, Avello M, Lei H, Mickanin C, Liu D, Ruffner H, Mao X, Ma Q, Zamponi R, Bouwmeester T, Finan PM, Kirschner MW, Porter JA, Serluca FC, Cong F. ZNRF3 promotes Wnt receptor turnover in an R-spondin-sensitive manner. Nature. 2012 Apr 29;485(7397):195-200. doi: 10.1038/nature11019. PMID:22575959 doi:10.1038/nature11019

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