nach oben

Pediatric Nephrology

Erschienen in:

01.09.2011 | Review

Glomerular basement membrane composition and the filtration barrier

verfasst von: Jeffrey H. Miner

Erschienen in: Pediatric Nephrology | Ausgabe 9/2011

Einloggen, um Zugang zu erhalten

Abstract

The glomerular basement membrane (GBM) is an especially thick basement membrane that contributes importantly to the kidney’s filtration barrier. The GBM derives from the fusion of separate podocyte and endothelial cell basement membranes during glomerulogenesis and consists primarily of laminin-521 (α5β2γ1), collagen α3α4α5(IV), nidogens-1 and -2, and agrin. Of these nine proteins, mutations in the genes encoding four of them (LAMB2, COL4A3, COL4A4, and COL4A5) cause glomerular disease in humans as well as in mice. Furthermore, mutation of a fifth (Lama5) gene in podocytes in mice causes proteinuria, nephrotic syndrome, and progression to renal failure. These results highlight the importance of the GBM for establishing and maintaining a properly functioning glomerular filtration barrier.

Farquhar MG (1975) Editorial: The primary glomerular filtration barrier–basement membrane or epithelial slits? Kidney Int 8:197–211CrossRef

St. John PL, Abrahamson DR (2001) Glomerular endothelial cells and podocytes jointly synthesize laminin-1 and -11 chains. Kidney Int 60:1037–1046CrossRef

Abrahamson DR (1985) Origin of the glomerular basement membrane visualized after in vivo labeling of laminin in newborn rat kidneys. J Cell Biol 100:1988–2000CrossRef

Abrahamson DR, Hudson BG, Stroganova L, Borza DB, St John PL (2009) Cellular origins of type IV collagen networks in developing glomeruli. J Am Soc Nephrol 20:1471–1479CrossRef

Schnabel E, Anderson JM, Farquhar MG (1990) The tight junction protein ZO-1 is concentrated along slit diaphragms of the glomerular epithelium. J Cell Biol 111:1255–1263CrossRef

Dressler GR (2006) The Cellular Basis of Kidney Development. Annu Rev Cell Dev Biol 22:509–529CrossRef

Deen WM, Lazzara MJ, Myers BD (2001) Structural determinants of glomerular permeability. Am J Physiol Ren Physiol 281:F579–F596CrossRef

Haraldsson B, Nystrom J, Deen WM (2008) Properties of the glomerular barrier and mechanisms of proteinuria. Physiol Rev 88:451–487CrossRef

Haraldsson B, Jeansson M (2009) Glomerular filtration barrier. Curr Opin Nephrol Hypertens 18:331–335CrossRef

10.

Aumailley M, Bruckner-Tuderman L, Carter WG, Deutzmann R, Edgar D, Ekblom P, Engel J, Engvall E, Hohenester E, Jones JC, Kleinman HK, Marinkovich MP, Martin GR, Mayer U, Meneguzzi G, Miner JH, Miyazaki K, Patarroyo M, Paulsson M, Quaranta V, Sanes JR, Sasaki T, Sekiguchi K, Sorokin LM, Talts JF, Tryggvason K, Uitto J, Virtanen I, von der Mark K, Wewer UM, Yamada Y, Yurchenco PD (2005) A simplified laminin nomenclature. Matrix Biol 24:326–332CrossRef

11.

Colognato H, Winkelmann DA, Yurchenco PD (1999) Laminin polymerization induces a receptor-cytoskeleton network. J Cell Biol 145:619–631CrossRef

12.

Miner JH, Patton BL, Lentz SI, Gilbert DJ, Snider WD, Jenkins NA, Copeland NG, Sanes JR (1997) The laminin alpha chains: expression, developmental transitions, and chromosomal locations of alpha1-5, identification of heterotrimeric laminins 8–11, and cloning of a novel alpha3 isoform. J Cell Biol 137:685–701CrossRef

13.

Miner JH, Sanes JR (1994) Collagen IV α3, α4, and α5 chains in rodent basal laminae: Sequence, distribution, association with laminins, and developmental switches. J Cell Biol 127:879–891CrossRef

14.

Hudson BG (2004) The molecular basis of Goodpasture and Alport syndromes: beacons for the discovery of the collagen IV family. J Am Soc Nephrol 15:2514–2527CrossRef

15.

Khoshnoodi J, Cartailler JP, Alvares K, Veis A, Hudson BG (2006) Molecular recognition in the assembly of collagens: terminal noncollagenous domains are key recognition modules in the formation of triple helical protomers. J Biol Chem 281:38117–38121CrossRef

16.

Harvey SJ, Zheng K, Sado Y, Naito I, Ninomiya Y, Jacobs RM, Hudson BG, Thorner PS (1998) Role of distinct type IV collagen networks in glomerular development and function. Kidney Int 54:1857–1866CrossRef

17.

Kalluri R, Shield CF III, Todd P, Hudson BG, Neilson EG (1997) Isoform switching of type IV collagen is developmentally arrested in X-linked Alport syndrome leading to increased susceptibility of renal basement membranes to endoproteolysis. J Clin Invest 99:2470–2478CrossRef

18.

Salmivirta K, Talts JF, Olsson M, Sasaki T, Timpl R, Ekblom P (2002) Binding of mouse nidogen-2 to basement membrane components and cells and its expression in embryonic and adult tissues suggest complementary functions of the two nidogens. Exp Cell Res 279:188–201CrossRef

19.

Bader BL, Smyth N, Nedbal S, Miosge N, Baranowsky A, Mokkapati S, Murshed M, Nischt R (2005) Compound genetic ablation of nidogen 1 and 2 causes basement membrane defects and perinatal lethality in mice. Mol Cell Biol 25:6846–6856CrossRef

20.

Groffen AJ, Ruegg MA, Dijkman H, van de Velden TJ, Buskens CA, van den Born J, Assmann KJ, Monnens LA, Veerkamp JH, van den Heuvel LP (1998) Agrin is a major heparan sulfate proteoglycan in the human glomerular basement membrane. J Histochem Cytochem 46:19–27CrossRef

21.

Kummer TT, Misgeld T, Sanes JR (2006) Assembly of the postsynaptic membrane at the neuromuscular junction: paradigm lost. Curr Opin Neurobiol 16:74–82CrossRef

22.

Harvey SJ, Jarad G, Cunningham J, Rops AL, van der Vlag J, Berden JH, Moeller MJ, Holzman LB, Burgess RW, Miner JH (2007) Disruption of glomerular basement membrane charge through podocyte-specific mutation of agrin does not alter glomerular permselectivity. Am J Pathol 171:139–152CrossRef

23.

Kestila M, Lenkkeri U, Mannikko M, Lamerdin J, McCready P, Putaala H, Ruotsalainen V, Morita T, Nissinen M, Herva R, Kashtan CE, Peltonen L, Holmberg C, Olsen A, Tryggvason K (1998) Positionally cloned gene for a novel glomerular protein–nephrin–is mutated in congenital nephrotic syndrome. Mol Cell 1:575–582CrossRef

24.

Machuca E, Benoit G, Antignac C (2009) Genetics of nephrotic syndrome: connecting molecular genetics to podocyte physiology. Hum Mol Genet 18:R185–R194CrossRef

25.

Farquhar MG (2006) The glomerular basement membrane: not gone, just forgotten. J Clin Invest 116:2090–2093CrossRef

26.

Goldberg S, Adair-Kirk TL, Senior RM, Miner JH (2010) Maintenance of glomerular filtration barrier integrity requires laminin alpha5. J Am Soc Nephrol 21:579–586CrossRef

27.

Barker DF, Hostikka SL, Zhou J, Chow LT, Oliphant AR, Gerken SC, Gregory MC, Skolnick MH, Atkin CL, Tryggvason K (1990) Identification of mutations in the COL4A5 collagen gene in Alport syndrome. Science 248:1224–1227CrossRef

28.

Kashtan CE (2000) Alport syndromes: phenotypic heterogeneity of progressive hereditary nephritis. Pediatr Nephrol 14:502–512CrossRef

29.

Kashtan CE, Kim Y (1992) Distribution of the α1 and α2 chains of collagen IV and of collagens V and VI in Alport syndrome. Kidney Int 42:115–126CrossRef

30.

Gould DB, Phalan FC, van Mil SE, Sundberg JP, Vahedi K, Massin P, Bousser MG, Heutink P, Miner JH, Tournier-Lasserve E, John SW (2006) Role of COL4A1 in small-vessel disease and hemorrhagic stroke. N Engl J Med 354:1489–1496CrossRef

31.

Plaisier E, Gribouval O, Alamowitch S, Mougenot B, Prost C, Verpont MC, Marro B, Desmettre T, Cohen SY, Roullet E, Dracon M, Fardeau M, Van Agtmael T, Kerjaschki D, Antignac C, Ronco P (2007) COL4A1 mutations and hereditary angiopathy, nephropathy, aneurysms, and muscle cramps. N Engl J Med 357:2687–2695CrossRef

32.

Zenker M, Tralau T, Lennert T, Pitz S, Mark K, Madlon H, Dotsch J, Reis A, Muntefering H, Neumann LM (2004) Congenital nephrosis, mesangial sclerosis, and distinct eye abnormalities with microcoria: an autosomal recessive syndrome. Am J Med Genet A 130:138–145CrossRef

33.

Zenker M, Aigner T, Wendler O, Tralau T, Muntefering H, Fenski R, Pitz S, Schumacher V, Royer-Pokora B, Wuhl E, Cochat P, Bouvier R, Kraus C, Mark K, Madlon H, Dotsch J, Rascher W, Maruniak-Chudek I, Lennert T, Neumann LM, Reis A (2004) Human laminin beta2 deficiency causes congenital nephrosis with mesangial sclerosis and distinct eye abnormalities. Hum Mol Genet 13:2625–2632CrossRef

34.

Noakes PG, Miner JH, Gautam M, Cunningham JM, Sanes JR, Merlie JP (1995) The renal glomerulus of mice lacking s-laminin/laminin β2: nephrosis despite molecular compensation by laminin β1. Nat Genet 10:400–406CrossRef

35.

Noakes PG, Gautam M, Mudd J, Sanes JR, Merlie JP (1995) Aberrant differentiation of neuromuscular junctions in mice lacking s-laminin/laminin β2. Nature 374:258–262CrossRef

36.

Libby RT, Lavallee CR, Balkema GW, Brunken WJ, Hunter DD (1999) Disruption of laminin beta2 chain production causes alterations in morphology and function in the CNS. J Neurosci 19:9399–9411CrossRef

37.

Hasselbacher K, Wiggins RC, Matejas V, Hinkes BG, Mucha B, Hoskins BE, Ozaltin F, Nurnberg G, Becker C, Hangan D, Pohl M, Kuwertz-Broking E, Griebel M, Schumacher V, Royer-Pokora B, Bakkaloglu A, Nurnberg P, Zenker M, Hildebrandt F (2006) Recessive missense mutations in LAMB2 expand the clinical spectrum of LAMB2-associated disorders. Kidney Int 70:1008–1012CrossRef

38.

Matejas V, Hinkes B, Alkandari F, Al-Gazali L, Annexstad E, Aytac MB, Barrow M, Blahova K, Bockenhauer D, Cheong HI, Maruniak-Chudek I, Cochat P, Dotsch J, Gajjar P, Hennekam RC, Janssen F, Kagan M, Kariminejad A, Kemper MJ, Koenig J, Kogan J, Kroes HY, Kuwertz-Broking E, Lewanda AF, Medeira A, Muscheites J, Niaudet P, Pierson M, Saggar A, Seaver L, Suri M, Tsygin A, Wuhl E, Zurowska A, Uebe S, Hildebrandt F, Antignac C, Zenker M (2010) Mutations in the human laminin beta2 (LAMB2) gene and the associated phenotypic spectrum. Hum Mutat 31:992–1002CrossRef

39.

Jarad G, Cunningham J, Shaw AS, Miner JH (2006) Proteinuria precedes podocyte abnormalities in Lamb2-/- mice, implicating the glomerular basement membrane as an albumin barrier. J Clin Invest 116:2272–2279CrossRef

Titel: Glomerular basement membrane composition and the filtration barrier
verfasst von: Jeffrey H. Miner
Publikationsdatum: 01.09.2011
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Nephrology / Ausgabe 9/2011
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-011-1785-1

Update Pädiatrie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Springer Medizin

Glomerular basement membrane composition and the filtration barrier

Abstract

Neu im Fachgebiet Pädiatrie

Kinder mit anhaltender Sinusitis profitieren häufig von Antibiotika

Neuer Typ-1-Diabetes bei Kindern am Wochenende eher übersehen

Neue Studienergebnisse zur Myopiekontrolle mit Atropin

Spinale Muskelatrophie: Neugeborenen-Screening lohnt sich

Update Pädiatrie

Springer Medizin

Abstract

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 9/2011

Kidney and urinary tract development: an apoptotic balancing act

BMP signaling in the nephron progenitor niche

Vesico-ureteric reflux: using mouse models to understand a common congenital urinary tract defect

Formation of atubular glomeruli in the developing kidney following chronic urinary tract obstruction

Poster Session

Wilms tumor—a renal stem cell malignancy?

Neu im Fachgebiet Pädiatrie

Kinder mit anhaltender Sinusitis profitieren häufig von Antibiotika

Neuer Typ-1-Diabetes bei Kindern am Wochenende eher übersehen

Neue Studienergebnisse zur Myopiekontrolle mit Atropin

Spinale Muskelatrophie: Neugeborenen-Screening lohnt sich

Update Pädiatrie