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{
    "metadata": {
        "accession": "cd11770",
        "entry_id": null,
        "type": "domain",
        "go_terms": null,
        "source_database": "cdd",
        "member_databases": null,
        "integrated": "IPR030642",
        "hierarchy": null,
        "name": {
            "name": "Src Homology 3 domain of Nephrocystin (or Nephrocystin-1)",
            "short": "SH3_Nephrocystin"
        },
        "description": [
            {
                "text": "<p>Nephrocystin contains an SH3 domain involved in signaling pathways that regulate cell adhesion and cytoskeletal organization. It is a protein that in humans is associated with juvenile nephronophthisis, an inherited kidney disease characterized by renal fibrosis that lead to chronic renal failure in children. It is localized in cell-cell junctions in renal duct cells, and is known to interact with Ack1, an activated Cdc42-associated kinase. SH3 domains are protein interaction domains that bind to proline-rich ligands with moderate affinity and selectivity, preferentially to PxxP motifs. They play versatile and diverse roles in the cell including the regulation of enzymes, changing the subcellular localization of signaling pathway components, and mediating the formation of multiprotein complex assemblies. [[cite:PUB00030871], [cite:PUB00129244], [cite:PUB00113512], [cite:PUB00129245], [cite:PUB00129246], [cite:PUB00129247], [cite:PUB00129248], [cite:PUB00129249], [cite:PUB00073418], [cite:PUB00128363], [cite:PUB00129250], [cite:PUB00129251], [cite:PUB00073417], [cite:PUB00129252], [cite:PUB00129253], [cite:PUB00129254], [cite:PUB00129255], [cite:PUB00129256], [cite:PUB00007145], [cite:PUB00085923], [cite:PUB00007124], [cite:PUB00085558], [cite:PUB00085556], [cite:PUB00085557], [cite:PUB00085559]]</p>",
                "llm": false,
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                "updated": false
            }
        ],
        "wikipedia": null,
        "literature": {
            "PUB00129253": {
                "PMID": 12006559,
                "ISBN": null,
                "volume": "277",
                "issue": "32",
                "year": 2002,
                "title": "Nephrocystin-conserved domains involved in targeting to epithelial cell-cell junctions, interaction with filamins, and establishing cell polarity.",
                "URL": null,
                "raw_pages": "29028-35",
                "medline_journal": "J Biol Chem",
                "ISO_journal": "J Biol Chem",
                "authors": [
                    "Donaldson JC",
                    "Dise RS",
                    "Ritchie MD",
                    "Hanks SK."
                ],
                "DOI_URL": null
            },
            "PUB00129256": {
                "PMID": 10665934,
                "ISBN": null,
                "volume": "11",
                "issue": "2",
                "year": 2000,
                "title": "Nephrocystin: gene expression and sequence conservation between human, mouse, and Caenorhabditis elegans.",
                "URL": null,
                "raw_pages": "270-282",
                "medline_journal": "J Am Soc Nephrol",
                "ISO_journal": "J Am Soc Nephrol",
                "authors": [
                    "Otto E",
                    "Kispert A",
                    "Schatzle S",
                    "Lescher B",
                    "Rensing C",
                    "Hildebrandt F."
                ],
                "DOI_URL": null
            },
            "PUB00030871": {
                "PMID": 15723349,
                "ISBN": null,
                "volume": "59",
                "issue": "2",
                "year": 2005,
                "title": "Solution NMR structure of the SH3 domain of human nephrocystin and analysis of a mutation-causing juvenile nephronophthisis.",
                "URL": null,
                "raw_pages": "347-55",
                "medline_journal": "Proteins",
                "ISO_journal": "Proteins",
                "authors": [
                    "le Maire A",
                    "Weber T",
                    "Saunier S",
                    "Broutin I",
                    "Antignac C",
                    "Ducruix A",
                    "Dardel F."
                ],
                "DOI_URL": "http://dx.doi.org/10.1002/prot.20344"
            },
            "PUB00129251": {
                "PMID": 17310360,
                "ISBN": null,
                "volume": "22",
                "issue": "6",
                "year": 2007,
                "title": "Nephrocystin and ciliary defects not only in the kidney?",
                "URL": null,
                "raw_pages": "765-9",
                "medline_journal": "Pediatr Nephrol",
                "ISO_journal": "Pediatr Nephrol",
                "authors": [
                    "von Schnakenburg C",
                    "Fliegauf M",
                    "Omran H."
                ],
                "DOI_URL": null
            },
            "PUB00129250": {
                "PMID": 18477472,
                "ISBN": null,
                "volume": "371",
                "issue": "4",
                "year": 2008,
                "title": "Nephrocystin-1 interacts directly with Ack1 and is expressed in human collecting duct.",
                "URL": null,
                "raw_pages": "877-82",
                "medline_journal": "Biochem Biophys Res Commun",
                "ISO_journal": "Biochem Biophys Res Commun",
                "authors": [
                    "Eley L",
                    "Moochhala SH",
                    "Simms R",
                    "Hildebrandt F",
                    "Sayer JA."
                ],
                "DOI_URL": null
            },
            "PUB00129247": {
                "PMID": 11261687,
                "ISBN": null,
                "volume": "16",
                "issue": "2",
                "year": 2001,
                "title": "New insights: nephronophthisis-medullary cystic kidney disease.",
                "URL": null,
                "raw_pages": "168-76",
                "medline_journal": "Pediatr Nephrol",
                "ISO_journal": "Pediatr Nephrol",
                "authors": [
                    "Hildebrandt F",
                    "Omram H."
                ],
                "DOI_URL": null
            },
            "PUB00085557": {
                "PMID": 16134966,
                "ISBN": null,
                "volume": "390",
                "issue": "Pt 3",
                "year": 2005,
                "title": "Specificity and versatility of SH3 and other proline-recognition domains: structural basis and implications for cellular signal transduction.",
                "URL": null,
                "raw_pages": "641-53",
                "medline_journal": "Biochem J",
                "ISO_journal": "Biochem. J.",
                "authors": [
                    "Li SS."
                ],
                "DOI_URL": "https://doi.org/10.1042/BJ20050411"
            },
            "PUB00128363": {
                "PMID": 18633336,
                "ISBN": null,
                "volume": "74",
                "issue": "9",
                "year": 2008,
                "title": "Jouberin localizes to collecting ducts and interacts with nephrocystin-1.",
                "URL": null,
                "raw_pages": "1139-49",
                "medline_journal": "Kidney Int",
                "ISO_journal": "Kidney Int",
                "authors": [
                    "Eley L",
                    "Gabrielides C",
                    "Adams M",
                    "Johnson CA",
                    "Hildebrandt F",
                    "Sayer JA."
                ],
                "DOI_URL": null
            },
            "PUB00007145": {
                "PMID": 11256992,
                "ISBN": null,
                "volume": "114",
                "issue": "Pt 7",
                "year": 2001,
                "title": "SH3 domains: complexity in moderation.",
                "URL": null,
                "raw_pages": "1253-63",
                "medline_journal": "J Cell Sci",
                "ISO_journal": "J. Cell. Sci.",
                "authors": [
                    "Mayer BJ."
                ],
                "DOI_URL": "http://jcs.biologists.org/cgi/content/abstract/114/7/1253"
            },
            "PUB00113512": {
                "PMID": 9326933,
                "ISBN": null,
                "volume": "17",
                "issue": "2",
                "year": 1997,
                "title": "A novel gene encoding an SH3 domain protein is mutated in nephronophthisis type 1.",
                "URL": null,
                "raw_pages": "149-53",
                "medline_journal": "Nat Genet",
                "ISO_journal": "Nat Genet",
                "authors": [
                    "Hildebrandt F",
                    "Otto E",
                    "Rensing C",
                    "Nothwang HG",
                    "Vollmer M",
                    "Adolphs J",
                    "Hanusch H",
                    "Brandis M."
                ],
                "DOI_URL": null
            },
            "PUB00129252": {
                "PMID": 16308564,
                "ISBN": null,
                "volume": "24",
                "issue": "24",
                "year": 2005,
                "title": "Phosphorylation by casein kinase 2 induces PACS-1 binding of nephrocystin and targeting to cilia.",
                "URL": null,
                "raw_pages": "4415-24",
                "medline_journal": "EMBO J",
                "ISO_journal": "EMBO J",
                "authors": [
                    "Schermer B",
                    "Hopker K",
                    "Omran H",
                    "Ghenoiu C",
                    "Fliegauf M",
                    "Fekete A",
                    "Horvath J",
                    "Kottgen M",
                    "Hackl M",
                    "Zschiedrich S",
                    "Huber TB",
                    "Kramer-Zucker A",
                    "Zentgraf H",
                    "Blaukat A",
                    "Walz G",
                    "Benzing T."
                ],
                "DOI_URL": null
            },
            "PUB00129244": {
                "PMID": 9361039,
                "ISBN": null,
                "volume": "6",
                "issue": "13",
                "year": 1997,
                "title": "A novel gene that encodes a protein with a putative src homology 3 domain is a candidate gene for familial juvenile nephronophthisis.",
                "URL": null,
                "raw_pages": "2317-23",
                "medline_journal": "Hum Mol Genet",
                "ISO_journal": "Hum Mol Genet",
                "authors": [
                    "Saunier S",
                    "Calado J",
                    "Heilig R",
                    "Silbermann F",
                    "Benessy F",
                    "Morin G",
                    "Konrad M",
                    "Broyer M",
                    "Gubler MC",
                    "Weissenbach J",
                    "Antignac C."
                ],
                "DOI_URL": null
            },
            "PUB00129249": {
                "PMID": 19755384,
                "ISBN": null,
                "volume": "18",
                "issue": "24",
                "year": 2009,
                "title": "Nephrocystin-1 and nephrocystin-4 are required for epithelial morphogenesis and associate with PALS1/PATJ and Par6.",
                "URL": null,
                "raw_pages": "4711-23",
                "medline_journal": "Hum Mol Genet",
                "ISO_journal": "Hum Mol Genet",
                "authors": [
                    "Delous M",
                    "Hellman NE",
                    "Gaude HM",
                    "Silbermann F",
                    "Le Bivic A",
                    "Salomon R",
                    "Antignac C",
                    "Saunier S."
                ],
                "DOI_URL": null
            },
            "PUB00073417": {
                "PMID": 16885411,
                "ISBN": null,
                "volume": "17",
                "issue": "9",
                "year": 2006,
                "title": "Nephrocystin specifically localizes to the transition zone of renal and respiratory cilia and photoreceptor connecting cilia.",
                "URL": null,
                "raw_pages": "2424-33",
                "medline_journal": "J Am Soc Nephrol",
                "ISO_journal": "J. Am. Soc. Nephrol.",
                "authors": [
                    "Fliegauf M",
                    "Horvath J",
                    "von Schnakenburg C",
                    "Olbrich H",
                    "Muller D",
                    "Thumfart J",
                    "Schermer B",
                    "Pazour GJ",
                    "Neumann HP",
                    "Zentgraf H",
                    "Benzing T",
                    "Omran H."
                ],
                "DOI_URL": "http://dx.doi.org/10.1681/ASN.2005121351"
            },
            "PUB00129248": {
                "PMID": 21357692,
                "ISBN": null,
                "volume": "286",
                "issue": "16",
                "year": 2011,
                "title": "Nephrocystin-4 regulates Pyk2-induced tyrosine phosphorylation of nephrocystin-1 to control targeting to monocilia.",
                "URL": null,
                "raw_pages": "14237-45",
                "medline_journal": "J Biol Chem",
                "ISO_journal": "J Biol Chem",
                "authors": [
                    "Liebau MC",
                    "Hopker K",
                    "Muller RU",
                    "Schmedding I",
                    "Zank S",
                    "Schairer B",
                    "Fabretti F",
                    "Hohne M",
                    "Bartram MP",
                    "Dafinger C",
                    "Hackl M",
                    "Burst V",
                    "Habbig S",
                    "Zentgraf H",
                    "Blaukat A",
                    "Walz G",
                    "Benzing T",
                    "Schermer B."
                ],
                "DOI_URL": null
            },
            "PUB00085559": {
                "PMID": 15853651,
                "ISBN": null,
                "volume": "6",
                "issue": "2",
                "year": 2005,
                "title": "SH3-like fold proteins are structurally conserved and functionally divergent.",
                "URL": null,
                "raw_pages": "143-50",
                "medline_journal": "Curr Protein Pept Sci",
                "ISO_journal": "Curr. Protein Pept. Sci.",
                "authors": [
                    "Kishan KV",
                    "Agrawal V."
                ],
                "DOI_URL": "https://doi.org/10.2174/1389203053545444"
            },
            "PUB00129245": {
                "PMID": 20652329,
                "ISBN": null,
                "volume": "26",
                "issue": "2",
                "year": 2011,
                "title": "Nephronophthisis.",
                "URL": null,
                "raw_pages": "181-94",
                "medline_journal": "Pediatr Nephrol",
                "ISO_journal": "Pediatr Nephrol",
                "authors": [
                    "Wolf MT",
                    "Hildebrandt F."
                ],
                "DOI_URL": null
            },
            "PUB00129254": {
                "PMID": 11493697,
                "ISBN": null,
                "volume": "98",
                "issue": "17",
                "year": 2001,
                "title": "Nephrocystin interacts with Pyk2, p130(Cas), and tensin and triggers phosphorylation of Pyk2.",
                "URL": null,
                "raw_pages": "9784-9",
                "medline_journal": "Proc Natl Acad Sci U S A",
                "ISO_journal": "Proc Natl Acad Sci U S A",
                "authors": [
                    "Benzing T",
                    "Gerke P",
                    "Hopker K",
                    "Hildebrandt F",
                    "Kim E",
                    "Walz G."
                ],
                "DOI_URL": null
            },
            "PUB00129246": {
                "PMID": 19118152,
                "ISBN": null,
                "volume": "20",
                "issue": "1",
                "year": 2009,
                "title": "Nephronophthisis: disease mechanisms of a ciliopathy.",
                "URL": null,
                "raw_pages": "23-35",
                "medline_journal": "J Am Soc Nephrol",
                "ISO_journal": "J Am Soc Nephrol",
                "authors": [
                    "Hildebrandt F",
                    "Attanasio M",
                    "Otto E."
                ],
                "DOI_URL": null
            },
            "PUB00073418": {
                "PMID": 19208653,
                "ISBN": null,
                "volume": "18",
                "issue": "9",
                "year": 2009,
                "title": "Essential role of nephrocystin in photoreceptor intraflagellar transport in mouse.",
                "URL": null,
                "raw_pages": "1566-77",
                "medline_journal": "Hum Mol Genet",
                "ISO_journal": "Hum. Mol. Genet.",
                "authors": [
                    "Jiang ST",
                    "Chiou YY",
                    "Wang E",
                    "Chien YL",
                    "Ho HH",
                    "Tsai FJ",
                    "Lin CY",
                    "Tsai SP",
                    "Li H."
                ],
                "DOI_URL": "http://dx.doi.org/10.1093/hmg/ddp068"
            },
            "PUB00007124": {
                "PMID": 11911877,
                "ISBN": null,
                "volume": "513",
                "issue": "1",
                "year": 2002,
                "title": "WW and SH3 domains, two different scaffolds to recognize proline-rich ligands.",
                "URL": null,
                "raw_pages": "30-7",
                "medline_journal": "FEBS Lett",
                "ISO_journal": "FEBS Lett.",
                "authors": [
                    "Macias MJ",
                    "Wiesner S",
                    "Sudol M."
                ],
                "DOI_URL": "http://dx.doi.org/10.1016/S0014-5793(01)03290-2"
            },
            "PUB00085556": {
                "PMID": 18508559,
                "ISBN": null,
                "volume": "13",
                "issue": null,
                "year": 2008,
                "title": "The SH3 domain--a family of versatile peptide- and protein-recognition module.",
                "URL": null,
                "raw_pages": "4938-52",
                "medline_journal": "Front Biosci",
                "ISO_journal": "Front. Biosci.",
                "authors": [
                    "Kaneko T",
                    "Li L",
                    "Li SS."
                ],
                "DOI_URL": "https://doi.org/10.2741/3053"
            },
            "PUB00085558": {
                "PMID": 12461825,
                "ISBN": null,
                "volume": "61",
                "issue": null,
                "year": 2002,
                "title": "How SH3 domains recognize proline.",
                "URL": null,
                "raw_pages": "211-68",
                "medline_journal": "Adv Protein Chem",
                "ISO_journal": "Adv. Protein Chem.",
                "authors": [
                    "Musacchio A."
                ],
                "DOI_URL": "https://doi.org/10.1016/S0065-3233(02)61006-X"
            },
            "PUB00129255": {
                "PMID": 10739664,
                "ISBN": null,
                "volume": "256",
                "issue": "1",
                "year": 2000,
                "title": "Crk-associated substrate p130(Cas) interacts with nephrocystin and both proteins localize to cell-cell contacts of polarized epithelial cells.",
                "URL": null,
                "raw_pages": "168-78",
                "medline_journal": "Exp Cell Res",
                "ISO_journal": "Exp Cell Res",
                "authors": [
                    "Donaldson JC",
                    "Dempsey PJ",
                    "Reddy S",
                    "Bouton AH",
                    "Coffey RJ",
                    "Hanks SK."
                ],
                "DOI_URL": null
            },
            "PUB00085923": {
                "PMID": 9241420,
                "ISBN": null,
                "volume": "26",
                "issue": null,
                "year": 1997,
                "title": "Modular peptide recognition domains in eukaryotic signaling.",
                "URL": null,
                "raw_pages": "259-88",
                "medline_journal": "Annu Rev Biophys Biomol Struct",
                "ISO_journal": "Annu Rev Biophys Biomol Struct",
                "authors": [
                    "Kuriyan J",
                    "Cowburn D."
                ],
                "DOI_URL": "https://doi.org/10.1146/annurev.biophys.26.1.259"
            }
        },
        "set_info": {
            "accession": "cl17036",
            "name": "SH3"
        },
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            },
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            "taxa": 2225
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}