Analysis Tools
renal/urinary system
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• at 8 weeks of age, (left) kidney weight-to-body weight is slight increased relative to littermate controls
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• increased excretion of most amino acids, with significant increases in threonine, tryptophan, valine, and alanine excretion is observed in 6-month old mice
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• mice exhibit mild glycosuria at 6 months of age
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• at 8 weeks, slight tubular cell hypertrophy is observed but interstitial nephritis or fibrosis is not apparent; accumulation of numerous crescent membranous structures in tubular epithelial cells, primarily adjacent to mitochondria is present at 6 weeks with accumulation of similar (smaller) structures observed at 9 months
• deformed mitochondria are found in tubular cells of mutants but not in controls
• accumulation of cytosolic amorphous substrates in proximal tubular cells is apparent at 6 months
• massive accumulation of p62- and ubiquitin-positive inclusion bodies is observed almost exclusively in proximal tubules at 9 months
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mortality/aging
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• all mice survive during observational period (<9 months)
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homeostasis/metabolism
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• no albuminuria or increased blood urea nitrogen level is observed in mice up to 9 months of age compared to littermate controls
• no phosphaturia is observed
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• in kidney cortex homogenates from 8-week old mice, conversion of microtubule-associated protein 1 light chain 3 (LC3-I) to LC3-II is suppressed, indicative of defective autophagy
• after ischemia-reperfusion injury, severely injured tubules with accumulation of tubular sediments and vacuolation in the cortex are observed, whereas injury severity is reduced in injured controls; conversion of LC-I to LC-II in cortex as measured by Western blot is significantly suppressed indicating autophagy deficiency
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• increased excretion of most amino acids, with significant increases in threonine, tryptophan, valine, and alanine excretion is observed in 6-month old mice
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• mice exhibit mild glycosuria at 6 months of age
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cellular
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• in kidney cortex homogenates from 8-week old mice, conversion of microtubule-associated protein 1 light chain 3 (LC3-I) to LC3-II is suppressed, indicative of defective autophagy
• after ischemia-reperfusion injury, severely injured tubules with accumulation of tubular sediments and vacuolation in the cortex are observed, whereas injury severity is reduced in injured controls; conversion of LC-I to LC-II in cortex as measured by Western blot is significantly suppressed indicating autophagy deficiency
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growth/size/body
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• at 8 weeks of age, (left) kidney weight-to-body weight is slight increased relative to littermate controls
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