Delayed chemokine receptor 1 blockade prolongs survival in collagen 4A3–deficient mice with Alport disease
Human Alport disease is caused by a lack of the α3-, 4-, or 5-chain of type IV collagen
(COL4A). Affected humans and COL4A3-deficient mice develop glomerulosclerosis and
progressive renal fibrosis in the presence of interstitial macrophages, but their contribution to
disease progression is under debate. This question was addressed by treating COL4A3-
deficient mice with BX471, an antagonist of chemokine receptor 1 (CCR1) that is known to
block interstitial leukocyte recruitment. Treatment with BX471 from weeks 6 to 10 of life …
(COL4A). Affected humans and COL4A3-deficient mice develop glomerulosclerosis and
progressive renal fibrosis in the presence of interstitial macrophages, but their contribution to
disease progression is under debate. This question was addressed by treating COL4A3-
deficient mice with BX471, an antagonist of chemokine receptor 1 (CCR1) that is known to
block interstitial leukocyte recruitment. Treatment with BX471 from weeks 6 to 10 of life …