Feto arlequin articulo

Páginas: 11 (2746 palabras) Publicado: 18 de mayo de 2011
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renal stem cells (Figure 1). The failure of  whole bone marrow to have this protective  effect (12) may be due to the extremely small  numbers of MSCs present in the bone marrow or the large numbers of inflammatory  cells infused in this preparation. It remains  unclear  whether  the  protective  effect  of MSCs requires them to leave the bone marrow and transit through the renal circulation or whether these cells can exert protective effects from distant sites. Identifying  the protective factor(s) and the signals that  prompt MSCs to secrete it should now be a  priority in our attempts to develop new therapeutic approaches for improving patient  outcomes following acute renal failure. Address  correspondence  to:  Lloyd  G.  Cantley,  Department  of  Internal Medicine, Yale University, 333 Cedar Street, Box  208029, New Haven, Connecticut 06520,  USA.  Phone:  (203)  785-7110;  Fax:  (203)  785-3756; E-mail: Lloyd.Cantley@yale.edu.
  1. Poulsom, R., et al. 2001. Bone marrow contributes  to renal parenchymal turnover and regeneration.    J. Pathol. 195:229–235.   2. Gupta, S., Verfaillie, C., Chmielewski, D., Kim, Y.,  and Rosenberg, M.E. 2002. A role for extrarenal cells in the regeneration following acute renal failure. Kidney Int. 62:1285–1290.   3. Lin, F., et al. 2003. Hematopoietic stem cells contribute to the regeneration of renal tubules after  renal ischemia-reperfusion injury in mice. J. Am. Soc. Nephrol. 14:1188–1199.   4. Kale, S., et al. 2003. Bone marrow stem cells contribute to repair of the ischemically injured renal  tubule.  J. Clin. Invest.  112:42–49.  doi:10.1172/JCI200317856.   5. Lagasse, E., et al. 2000. Purified hematopoietic stem  cells can differentiate into hepatocytes in vivo. Nat. Med. 6:1229–1234.   6. Krause, D.S., et al. 2001. Multi-organ, multi-lineage  engraftment by a single bone marrow-derived stem  cell. Cell. 105:369–377.   7. Herzog, E.L., Chai, L., and Krause, D.S. 2003. Plasticity of marrow-derived stem cells [review]. Blood.  102:3483–3493.  8. Morigi, M., et al. 2004. Mesenchymal stem cells  are renotropic, helping to repair the kidney and  improve function in acute renal failure. J. Am. Soc. Nephrol. 15:1794–1804.   9. Herrera, M.B., et al. 2004. Mesenchymal stem cells  contribute to the renal repair of acute tubular epithelial injury. Int. J. Mol. Med. 14:1035–1041.   10. Duffield, J. 2005. Restoration of tubular epithelial cells during repair of the postischemic kidney occurs    independently of bone marrow–derived stem cells.  J. Clin. Invest. 115:1743–1755. doi:10.1172/JCI22593.   11. Togel, F., et al. 2005. Administered mesenchymal  stem cells protect against ischemic acute renal failure through differentiation-independent mechanisms.  Am. J. Physiol. Renal Physiol.  doi:10.1152/ ajprenal.00007.2005.  12. Lin, F., Moran, A., and Igarashi, P. 2005. Intrarenal  cells, not bone marrow–derived cells, are the major    source for regeneration in postischemic kidney.  J. Clin. Invest. 115:1756–1764. doi:10.1172/JCI23015.   13. Javazon, E.H., Beggs, K.J., and Flake, A.W. 2004.  Mesenchymal stem cells: paradoxes of passaging.  Exp. Hematol. 32:414–425.   14. Oliver, J.A., Maarouf, O., Cheema, F.H., Martens, T.P., and Al-Awqati, Q. 2004. The renal papilla is  a niche for adult kidney stem cells. J. Clin. Invest.  114:795–804. doi:10.1172/JCI200420921.

Harlequin ichthyosis unmasked: a defect of lipid transport
Alain Hovnanian
Department of Medical Genetics and INSERM U563, Purpan Hospital, Toulouse, France.

Harlequin ichthyosis (HI) — the most severe form of keratinizing disorders, often lethal in the neonatal period — ischaracterized by a profound thickening of the keratin skin layer, a dense “armor”-like scale that covers the body, and contraction abnormalities of the eyes, ears, and mouth. In this issue of the JCI, Akiyama et al. report that mutations in ABCA12 caused defective lipid transport that significantly impacted normal development of the...
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