Analysis Tools
digestive/alimentary system
|
• mice show differences in the proportion of and type of bacteria species in stool, with an increase of firmicutes relative to Bacteroidetes (strongest in Blautia genus containing the families Ruminococcaceae and Lachnospiraceae, then Adlercreutzia genus, Bilophilia genus, and AF12 genus and a decrease in Bifidobacterium genus); microbiome changes resemble those seen in microbiome disbalance in metabolic diseases like diabetes
|
growth/size/body
|
• 52-week old mice show an increase in body weight
|
kidney cyst
(
J:293423
)
|
• mice fed a lysine-enriched diet show cystic change in the kidney accompanied with increased renal tubular vacuolation
|
adipose tissue
|
• aged mice show adipocyte atrophy in the kidney
|
|
• aged mice show a greater degree of inflammation in epididymal white adipose tissue, often forming crown-like structures
|
hematopoietic system
|
• 3-fold increase of macrophages in kidneys of aged mice
• mice fed a lysine-enriched diet show almost a 2.5-fold increase in the number of macrophages in the kidney compared to mutant mice fed a normal diet
|
homeostasis/metabolism
proteinosis
(
J:293423
)
|
• mice fed a lysine-enriched diet show tubular proteinosis
|
|
• mice exhibit imbalanced lipid metabolism in the kidney leading to an accumulation of lipids in the kidney
|
immune system
|
• aged mice show a greater degree of inflammation in epididymal white adipose tissue, often forming crown-like structures
|
|
• 3-fold increase of macrophages in kidneys of aged mice
• mice fed a lysine-enriched diet show almost a 2.5-fold increase in the number of macrophages in the kidney compared to mutant mice fed a normal diet
|
|
• 52-week old mice show an increase of interstitial mononuclear cell infiltrate in the kidney, indicating inflammation
• mice fed a lysine-enriched diet for 20 weeks show exacerbated interstitial mononuclear cell infiltrate
|
liver/biliary system
|
• micro- and macrovesicular steatosis in the liver of aged mice
|
renal/urinary system
|
• lipid accumulation in the kidney of aged mice
• kidney shows an 11-fold increase in glutarate levels and an increase in indoleacrylic acid levels
• however, no changes were seen in acetylcarnitine, 3-hydroxyglutarate, glutarylcarnitine, or glutarylglycine in the kidney
• mice fed a lysine-enriched diet show a further increase in lipid accumulation in the kidney
• mice fed a lysine-enriched diet for 20 weeks show aggravated kidney changes compared to controls, with exacerbated interstitial mononuclear cell infiltrate, medullary tubule mineralization, tubular proteinosis, tubule dilation, and cystic change accompanied with increased renal tubular vacuolation
|
kidney cyst
(
J:293423
)
|
• mice fed a lysine-enriched diet show cystic change in the kidney accompanied with increased renal tubular vacuolation
|
|
• kidneys show an increase in number of vesicles in cytoplasm of renal tubular epithelial cells in a subset of mice
|
|
• mice fed a lysine-enriched diet show medullary tubule mineralization
|
|
• mice fed a lysine-enriched diet show tubule dilation
|
|
• mice show imbalanced lipid and acylcarnitine metabolism in kidney
• antibiotic treatment to clear the gut microflora eliminates the metabolic differences
|
|
• 52-week old mice show an increase of interstitial mononuclear cell infiltrate in the kidney, indicating inflammation
• mice fed a lysine-enriched diet for 20 weeks show exacerbated interstitial mononuclear cell infiltrate
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| glutaric acidemia type 3 | DOID:0112246 |
OMIM:231690 |
J:293423 | |
