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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/11/26 00:36:57」(JST)

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Nephrin is a protein necessary for the proper functioning of the renal filtration barrier. The renal filtration barrier consists of fenestrated endothelial cells, the glomerular basement membrane, and the podocytes of epithelial cells. Nephrin is a transmembrane protein that is a structural component of the slit diaphragm.^[1] They are present on the tips of the podocytes and maintain the normal relationship between the basment membrane and the podocytes of the epithelial cells.

A defect in the gene for nephrin, NPHS1, is associated with congenital nephrotic syndrome of the Finnish type and causes massive amounts of protein to be leaked into the urine, or proteinuria. Nephrin is also required for cardiovascular development.^[2]

Interactions

Nephrin has been shown to interact with:

CASK,^[3]
CD2AP,^[3]
CDH3^[3] and
CTNND1,^[3]
FYN,^[4]^[5]
KIRREL,^[6]^[7] and
NPHS2.^[8]

References

^ "Entrez Gene: NPHS1 nephrosis 1, congenital, Finnish type (nephrin)".
^ Wagner N, Morrison H, Pagnotta S, Michiels JF, Schwab Y, Tryggvason K, Schedl A, Wagner KD (2011-03-29). "The podocyte protein nephrin is required for cardiac vessel formation.". Human Molecular Genetics 20 (11): 2182–94. doi:10.1093/hmg/ddr106. PMID 21402589.
^ ^a ^b ^c ^d Lehtonen S, Lehtonen E, Kudlicka K, Holthöfer H, Farquhar MG (Sep 2004). "Nephrin forms a complex with adherens junction proteins and CASK in podocytes and in Madin-Darby canine kidney cells expressing nephrin". Am. J. Pathol. 165 (3): 923–36. doi:10.1016/S0002-9440(10)63354-8. PMC 1618613. PMID 15331416.
^ Lahdenperä J, Kilpeläinen P, Liu XL, Pikkarainen T, Reponen P, Ruotsalainen V, Tryggvason K (Aug 2003). "Clustering-induced tyrosine phosphorylation of nephrin by Src family kinases". Kidney Int. 64 (2): 404–13. doi:10.1046/j.1523-1755.2003.00097.x. PMID 12846735.
^ Verma R, Wharram B, Kovari I, Kunkel R, Nihalani D, Wary KK, Wiggins RC, Killen P, Holzman LB (Jun 2003). "Fyn binds to and phosphorylates the kidney slit diaphragm component Nephrin". J. Biol. Chem. 278 (23): 20716–23. doi:10.1074/jbc.M301689200. PMID 12668668.
^ Liu G, Kaw B, Kurfis J, Rahmanuddin S, Kanwar YS, Chugh SS (Jul 2003). "Neph1 and nephrin interaction in the slit diaphragm is an important determinant of glomerular permeability". J. Clin. Invest. 112 (2): 209–21. doi:10.1172/JCI18242. PMC 164293. PMID 12865409.
^ Gerke P, Huber TB, Sellin L, Benzing T, Walz G (Apr 2003). "Homodimerization and heterodimerization of the glomerular podocyte proteins nephrin and NEPH1". J. Am. Soc. Nephrol. 14 (4): 918–26. doi:10.1097/01.ASN.0000057853.05686.89. PMID 12660326.
^ Schwarz K, Simons M, Reiser J, Saleem MA, Faul C, Kriz W, Shaw AS, Holzman LB, Mundel P (Dec 2001). "Podocin, a raft-associated component of the glomerular slit diaphragm, interacts with CD2AP and nephrin". J. Clin. Invest. 108 (11): 1621–9. doi:10.1172/JCI12849. PMC 200981. PMID 11733557.

External links

nephrin at the US National Library of Medicine Medical Subject Headings (MeSH)

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UpToDate Contents

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1. 巣状分節性糸球体硬化症の疫学、分類、および病因 epidemiology classification and pathogenesis of focal segmental glomerulosclerosis
2. 先天性乳児ネフローゼ症候群 congenital and infantile nephrotic syndrome
3. 糸球体足細胞のバイオロジー biology of glomerular podocytes
4. 糖尿病性腎症の概要 overview of diabetic nephropathy
5. 糸球体の免疫障害の機序 mechanisms of immune injury of the glomerulus

English Journal

Podocyte-specific Nox4 deletion affords renoprotection in a mouse model of diabetic nephropathy.

Jha JC1,2, Thallas-Bonke V1, Banal C1, Gray SP1, Chow BS1, Ramm G3, Quaggin SE4, Cooper ME1,2, Schmidt HH5, Jandeleit-Dahm KA6,7.
Diabetologia.Diabetologia.2016 Feb;59(2):379-89. doi: 10.1007/s00125-015-3796-0. Epub 2015 Oct 28.
AIMS/HYPOTHESIS: Changes in podocyte morphology and function are associated with albuminuria and progression of diabetic nephropathy. NADPH oxidase 4 (NOX4) is the main source of reactive oxygen species (ROS) in the kidney and Nox4 is upregulated in podocytes in response to high glucose. We assessed
PMID 26508318

FK506 reduces albuminuria through improving podocyte nephrin and podocin expression in diabetic rats.

Qi XM1, Wang J1, Xu XX1, Li YY1, Wu YG2.
Inflammation research : official journal of the European Histamine Research Society ... [et al.].Inflamm Res.2016 Feb;65(2):103-14. doi: 10.1007/s00011-015-0893-y. Epub 2015 Nov 13.
OBJECTIVE AND DESIGN: Several works in the setting of early experimental diabetic nephropathy using anti-inflammatory drugs, such as the calcineurin inhibitor FK506, have shown prevention of the development or amelioration of renal injury including proteinuria. The exact mechanisms by which anti-inf
PMID 26566632

Role of nephrin in podocyte injury induced by angiotension II.

Yu S1.
Journal of receptor and signal transduction research.J Recept Signal Transduct Res.2016 Feb;36(1):1-5. doi: 10.3109/10799893.2014.963872. Epub 2014 Sep 29.
OBJECTIVE: To investigate the function of nephrin in podocytes and its relation to proteinuria in kidney diseases, and to study more clearly theoretical basis for the molecular mechanism of losartan anti-proteinuria and the special beneficial effects of losartan on podocyte injury.METHODS: Experimen
PMID 25264225

Japanese Journal

平成22年度横浜市立大学医学研究奨励賞受賞研究糸球体濾過膜の維持機構異常から考える糸球体疾患の分子病態

廣瀬智威
横浜医学 63(1), 53-60, 2012-01-00
NAID 40019374524

Dexamethasone increases the phosphorylation of nephrin in cultured podocytes

OHASHI Teiko,UCHIDA Keiko,UCHIDA Shinichi,SASAKI Sei,NITTA Kosaku
Clinical and experimental nephrology 15(5), 688-693, 2011-10-01
NAID 10030340996

Injection of rabbit polyclonal antibodies induced by genetic immunization with rat nephrin cDNA causes massive proteinuria in the rat: a new model of nephrotic syndrome

Okamoto Tomoko,Kamata Kouju,Naito Shokichi [他]
The Kitasato medical journal 41(2), 115-122, 2011-09-00
NAID 40019009576

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Nephrosis 1, congenital, Finnish type (nephrin)
Identifiers
Symbols	NPHS1 ; CNF; NPHN; nephrin
External IDs	OMIM: 602716 MGI: 1859637 HomoloGene: 20974 GeneCards: NPHS1 Gene
Gene ontology
Molecular function	• protein binding; • myosin binding;
Cellular component	• intracellular; • plasma membrane; • integral component of plasma membrane; • slit diaphragm; • cell projection; • extracellular exosome;
Biological process	• cell adhesion; • JNK cascade; • skeletal muscle tissue development; • myoblast fusion; • excretion; • positive regulation of actin filament polymerization; • glomerular basement membrane development; • regulation of excretion; • glomerular visceral epithelial cell development;
	Sources: Amigo / QuickGO
Orthologs
	v; t; e;