Article
A human-mouse chimera of the alpha3alpha4alpha5(IV) collagen protomer rescues the renal phenotype in Col4a3-/- Alport mice.
The American journal of pathology - 1 Oct 2003
Heidet Laurence, Borza Dorin-Bogdan, Jouin Mélanie, Sich Mireille, Mattei Marie-Geneviève, Sado Yoshikazu, Hudson Billy G, Hastie Nicholas, Antignac Corinne, Gubler Marie-Claire
Abstract excerpt
Collagen IV is a major structural component of basement membranes. In the glomerular basement membrane (GBM) of the kidney, the alpha3, alpha4, and alpha5(IV) collagen chains form a distinct network that is essential for the long-term stability of the glomerular filtration barrier, and is absent in most patients affected with Alport syndrome, a progressive inherited nephropathy associated with mutation in COL4A3,...
Topics
- Animals
- Autoantibodies
- Autoantigens
- Basement Membrane
- Chimera
- Collagen Type IV
- Extracellular Matrix Proteins
- Humans
- Kidney
- Mice
- Mice, Knockout
- Mice, Transgenic
- Nephritis, Hereditary