Kidney development and disease in the zebrafish.

Unraveling the molecular pathogenesis of human disease presents many experimental challenges, not the least of which is that experiments on humans are generally frowned upon. Model organisms, including the zebrafish, allow for experimental analysis of gene function and the detailed characterization of disease processes. Zebrafish have matured as a vertebrate model organism now that genetic tools for targeted "knockdowns" and unbiased mutagenesis approaches are in hand. The fish larval pronephros is a relevant kidney in which to pursue many aspects of human kidney development and disease. This short review outlines recent progress in applying the zebrafish pronephros to issues of human health and development.

[1]  K. R. Spring,et al.  Bending the MDCK Cell Primary Cilium Increases Intracellular Calcium , 2001, The Journal of Membrane Biology.

[2]  Nektarios Tavernarakis,et al.  Molecular modeling of mechanotransduction in the nematode Caenorhabditis elegans. , 1997, Annual review of physiology.

[3]  N. Heintz,et al.  To beat or not to beat: roles of cilia in development and disease. , 2003, Human molecular genetics.

[4]  G. Pazour,et al.  Chlamydomonas IFT88 and Its Mouse Homologue, Polycystic Kidney Disease Gene Tg737, Are Required for Assembly of Cilia and Flagella , 2000, The Journal of cell biology.

[5]  G. Pazour,et al.  Polycystin-2 localizes to kidney cilia and the ciliary level is elevated in orpk mice with polycystic kidney disease , 2002, Current Biology.

[6]  W. Rottbauer,et al.  Growth and function of the embryonic heart depend upon the cardiac-specific L-type calcium channel alpha1 subunit. , 2001, Developmental cell.

[7]  M. Barr,et al.  An autosomal recessive polycystic kidney disease gene homolog is involved in intraflagellar transport in C. elegans ciliated sensory neurons , 2001, Current Biology.

[8]  A. Majumdar,et al.  The zebrafish floating head mutant demonstrates podocytes play an important role in directing glomerular differentiation. , 2000, Developmental biology.

[9]  M. Fishman,et al.  Vessel patterning in the embryo of the zebrafish: guidance by notochord. , 1997, Developmental biology.

[10]  B. L. Roman,et al.  Isolation and expression analysis of three zebrafish angiopoietin genes , 2001, Developmental dynamics : an official publication of the American Association of Anatomists.

[11]  A. Schier,et al.  Mutations affecting the formation and function of the cardiovascular system in the zebrafish embryo. , 1996, Development.

[12]  J. Bard,et al.  The kidney : from normal development to congenital disease , 2003 .

[13]  A. Fry.  The Nek2 protein kinase: a novel regulator of centrosome structure , 2002, Oncogene.

[14]  P. Scambler,et al.  Evolving concepts in human renal dysplasia. , 2004, Journal of the American Society of Nephrology : JASN.

[15]  Jing Zhou,et al.  Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells , 2003, Nature Genetics.

[16]  J. Calvet,et al.  The genetics and physiology of polycystic kidney disease. , 2001, Seminars in nephrology.

[17]  Andreas Reimann,et al.  A transcriptional network in polycystic kidney disease , 2004, The EMBO journal.

[18]  B. Yoder,et al.  Polaris, a protein involved in left-right axis patterning, localizes to basal bodies and cilia. , 2001, Molecular biology of the cell.

[19]  L. Guay-Woodford,et al.  The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia. , 2002, Journal of the American Society of Nephrology : JASN.

[20]  E. Marshall,et al.  THE GLOMERULAR DEVELOPMENT OF THE VERTEBRATE KIDNEY IN RELATION TO HABITAT , 1930 .

[21]  I. Drummond.  Making a zebrafish kidney: a tale of two tubes. , 2003, Trends in cell biology.

[22]  M. Fishman,et al.  Endothelial Signaling in Kidney Morphogenesis A Role for Hemodynamic Forces , 2002, Current Biology.

[23]  G. ELLIOT SMITH,et al.  Studies on the Structure and Development of Vertebrates , 1930, Nature.

[24]  P. Tytler,et al.  Morphology of the pronephros of the juvenile brown trout, Salmo trutta , 1988, Journal of morphology.

[25]  O. Gribouval,et al.  Podocin localizes in the kidney to the slit diaphragm area. , 2002, The American journal of pathology.

[26]  K. Dewar,et al.  A defect in a novel Nek-family kinase causes cystic kidney disease in the mouse and in zebrafish , 2002, Development.

[27]  N. Hopkins,et al.  vhnf1, the MODY5 and familial GCKD-associated gene, regulates regional specification of the zebrafish gut, pronephros, and hindbrain. , 2001, Genes & development.

[28]  M. Kretzler,et al.  The glomerular slit diaphragm is a modified adherens junction. , 2000, Journal of the American Society of Nephrology : JASN.

[29]  J. Dowling,et al.  Small molecule developmental screens reveal the logic and timing of vertebrate development. , 2000, Proceedings of the National Academy of Sciences of the United States of America.

[30]  D A Kane,et al.  Characterization of zebrafish mutants with defects in embryonic hematopoiesis. , 1996, Development.

[31]  J. T. Wearn,et al.  OBSERVATIONS ON THE COMPOSITION OF GLOMERULAR URINE, WITH PARTICULAR REFERENCE TO THE PROBLEM OF REABSORPTION IN THE RENAL TUBULES , 1924 .

[32]  K. Tryggvason,et al.  Nephrin is specifically located at the slit diaphragm of glomerular podocytes. , 1999, Proceedings of the National Academy of Sciences of the United States of America.

[33]  C. Bugg,et al.  Polaris, a protein disrupted in orpk mutant mice, is required for assembly of renal cilium. , 2002, American journal of physiology. Renal physiology.

[34]  J. Grantham.  The kidney: from normal development to congenital disease , 2003 .

[35]  A. Schier,et al.  Early development of the zebrafish pronephros and analysis of mutations affecting pronephric function. , 1998, Development.

[36]  M. Cooper,et al.  Role of nephrin in renal disease including diabetic nephropathy. , 2002, Seminars in nephrology.

[37]  J. Forrest,et al.  Mechanism of active chloride secretion by shark rectal gland: role of Na-K-ATPase in chloride transport. , 1977, The American journal of physiology.

[38]  A. Schier,et al.  A genetic screen for mutations affecting embryogenesis in zebrafish. , 1996, Development.

[39]  A. Majumdar,et al.  Podocyte differentiation in the absence of endothelial cells as revealed in the zebrafish avascular mutant, cloche. , 1999, Developmental genetics.

[40]  D. Hall,et al.  The Caenorhabditis elegans autosomal dominant polycystic kidney disease gene homologs lov-1 and pkd-2 act in the same pathway , 2001, Current Biology.

[41]  C. Kimmel,et al.  Stages of embryonic development of the zebrafish , 1995, Developmental dynamics : an official publication of the American Association of Anatomists.

[42]  T. Strachan,et al.  Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination , 2003, Nature Genetics.

[43]  A. Schier,et al.  Mutations affecting development of zebrafish digestive organs. , 1996, Development.

[44]  T. Strachan,et al.  Expression analyses and interaction with the anaphase promoting complex protein Apc2 suggest a role for inversin in primary cilia and involvement in the cell cycle. , 2002, Human molecular genetics.

[45]  E. L. Potter.  Normal and abnormal development of the kidney , 1972 .

[46]  Mark S. Miller,et al.  A genetic screen in zebrafish identifies cilia genes as a principal cause of cystic kidney , 2004, Development.