Alport syndrome(AS) is a hereditary nephritis caused by mutations in COL4A3,COL4A4 or COL4A5 encoding the type IV collagen α3,α4,and α5 chains,which are major components of the glomerular basement membrane.About 20 years have passed since COL4A3,COL4A4,and COL4A5 were identified and the first Alport mouse model was developed using a knockout approach.The phenotype of Alport mice is similar to that of Alport patients,including characteristic thickening and splitting of the glomerular basement membrane.Alport mice have been widely used to study the pathogenesis of AS and to develop effective therapies.In this review,the newer therapies for AS,such as pharmacological interventions,genetic approaches and stem cell therapies,are discussed.Although some stem cel therapies have been demonstrated to slow the rena disease progression in Alport mice,these therapies demand continual refinement as research advances.In terms of the pharmacological drugs,angiotensin-converting enzyme inhibitors have been shown to be effective in Alport mice.Novel therapies that can provide a better outcome or lead to a cure are still awaited.
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机译：Neural activity in the periaqueductal gray and other specific subcortical structures is enhanced when a selective serotonin reuptake inhibitor selectively prevents seizure-induced sudden death in the DBA/1 mouse model of sudden unexpected death in epilepsy
