Small but powerful is an apt description of the human kidney. Roughly the size of your fists, your kidneys clear the waste from your blood and produce urine. This is orchestrated by filtering units called nephrons, with each kidney containing roughly a million of them. Too few nephrons and your kidneys can wear out resulting in kidney failure, as in children with renal hypoplasia. In newborns, a mutation in the gene OSR1 was linked to smaller kidneys with impaired function. Investigating this further in mice researchers found that the OSR1 protein works with another protein WT1 to manage kidney development. Mice with mutant OSR1 (middle) or WT1 (right) showed normal kidney development. However mice with mutant OSR1 and WT1 showed defects in the development of their nephrons (left). This was observed as less branching of structures called ureteric buds (green). These insights provide clues to what's happening in renal hypoplasia.
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