Laminin-521 Protein Therapy for Glomerular Basement Membrane and Podocyte Abnormalities in a Model of Pierson Syndrome

Background Laminin α5β2γ1 (LM-521) is a major component of the GBM. Mutations in LAMB2 that prevent LM-521 synthesis and/or secretion cause Pierson syndrome, a rare congenital nephrotic syndrome with diffuse mesangial sclerosis and ocular and neurologic defects. Because the GBM is uniquely accessibl...

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Enregistré dans:
Détails bibliographiques
Publié dans:J Am Soc Nephrol
Auteurs principaux: Lin, Meei-Hua, Miller, Joseph B., Kikkawa, Yamato, Suleiman, Hani Y., Tryggvason, Karl, Hodges, Bradley L., Miner, Jeffrey H.
Format: Artigo
Langue:Inglês
Publié: American Society of Nephrology 2018
Sujets:
Basic Research
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC5967757/
https://ncbi.nlm.nih.gov/pubmed/29472414
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1681/ASN.2017060690
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