ArticleProceedings of the National Academy of Sciences of the United States of America2011
Forced expression of laminin beta1 in podocytes prevents nephrotic syndrome in mice lacking laminin beta2, a model for Pierson syndrome.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
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Who cites it
31 citing papers in PubMed, 55 citations in OpenAlex.
- Identification of Genes Associated with Familial Focal Segmental Glomerulosclerosis Through Transcriptomics and In Silico Analysis, IncludingInternational journal of molecular sciences · 2024Article
- Experimental models for preclinical research in kidney disease.Zoological research · 2024Review
- LAMB2 gene: broad clinical spectrum in Pierson syndrome.CEN case reports · 2024Article
- Characterization of glomerular basement membrane components within pediatric glomerular diseases.Clinical kidney journal · 2024Article
- An organ-derived extracellular matrix triggers in situ kidney regeneration in a preclinical model.NPJ Regenerative medicine · 2022Article
- Neurological involvement in monogenic podocytopathies.Pediatric nephrology (Berlin, Germany) · 2021Review
- Tissue-specific parameters for the design of ECM-mimetic biomaterials.Acta biomaterialia · 2021Review
- Laminin β2 variants associated with isolated nephropathy that impact matrix regulation.JCI insight · 2021Article
- Complexities of the glomerular basement membrane.Nature reviews. Nephrology · 2021Review
- A deletion in the N-terminal polymerizing domain of laminin β2 is a new mouse model of chronic nephrotic syndrome.Kidney international · 2020Article
- Development of a novel prostate apoptosis response-4 (Par-4) protein entity with an extended duration of action for therapeutic treatment of cancer.Protein engineering, design & selection : PEDS · 2019Article
- A Novel Homozygous Truncating Mutation inFrontiers in medicine · 2019Article
- Alport syndrome and Pierson syndrome: Diseases of the glomerular basement membrane.Matrix biology : journal of the International Society for Matrix Biology · 2018Review
- Recent advances of animal model of focal segmental glomerulosclerosis.Clinical and experimental nephrology · 2018Review
- Laminin-521 Protein Therapy for Glomerular Basement Membrane and Podocyte Abnormalities in a Model of Pierson Syndrome.Journal of the American Society of Nephrology : JASN · 2018Article
- Repairing the GBM Step by Step.Journal of the American Society of Nephrology : JASN · 2018Article
- Pathogenicity of a Human LamininJournal of the American Society of Nephrology : JASN · 2018Article
- Basement Membrane Defects in Genetic Kidney Diseases.Frontiers in pediatrics · 2018Review
- Article
- Cell Receptor-Basement Membrane Interactions in Health and Disease: A Kidney-Centric View.Current topics in membranes · 2015Review
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Authors and funding
4 authors at 2 institutions in 2 countries.
Funding
Abstract
Pierson syndrome is a congenital nephrotic syndrome with ocular and neurological defects caused by mutations in LAMB2, the gene encoding the basement membrane protein laminin β2 (Lamβ2). It is the kidney glomerular basement membrane (GBM) that is defective in Pierson syndrome, as Lamβ2 is a component of laminin-521 (LM-521; α5β2γ1), the major laminin in the mature GBM. In both Pierson syndrome and the Lamb2(-/-) mouse model for this disease, laminin β1 (Lamβ1), a structurally similar homolog of Lamβ2, is marginally increased in the GBM, but it fails to fully compensate for the loss of Lamβ2, leading to the filtration barrier defects and nephrotic syndrome. Here we generated several lines of Lamβ1 transgenic mice and used them to show that podocyte-specific Lamβ1 expression in Lamb2(-/-) mice abrogates the development of nephrotic syndrome, correlating with a greatly extended lifespan. In addition, the more Lamβ1 was expressed, the less urinary albumin was excreted. Transgenic Lamβ1 expression increased the level of Lamα5 in the GBM of rescued mice, consistent with the desired increased deposition of laminin-511 (α5β1γ1) trimers. Ultrastructural analysis revealed occasional knob-like subepithelial GBM thickening but intact podocyte foot processes in aged rescued mice. These results suggest the possibility that up-regulation of LAMB1 in podocytes, should it become achievable, would likely lessen the severity of nephrotic syndrome in patients carrying LAMB2 mutations.
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