Analysis Tools
growth/size/body
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• females show a 30% difference in body weight from wild-type mice at about 1 year of age
• however, food intake remains constant
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weight loss
(
J:237040
)
|
• mice fed a high protein (HP) diet or a precursor-enriched (PE) diet comprised of increased levels of precursor amino acids of propionate pathway metabolites exhibit a rapid weight loss, with more pronounced weight loss on the PE diet
• mice fed a high protein diet and treated with cobalamin show a slight protective effect, with a delay in weight loss, although increases in metabolite levels still occur
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• mice show significant growth retardation after the age of about 100 days in females and about 150 days in males
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homeostasis/metabolism
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• propionylcarnitine (normalized to acetylcarnitine) in blood is constantly elevated
• mice fed a HP or PE diet show further elevations in propionylcarnitine levels
|
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• mice exhibit elevated glycine levels in blood
• however, other amino acid levels are mostly unchanged
|
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• mice fed a HP or PE diet show increased ammonia levels, indicative of metabolic crisis induction
• however, mice fed a regular chow diet exhibit normal ammonia levels
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• increase in plasma urea
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• mice exhibit increased levels of odd chain fatty acids in plasma, including the 17-carbon chain length fatty acid
• however, levels of even chain fatty acids are normal
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• mice exhibit an elevation of odd chain length sphingoid bases in plasma and tissues
• however, levels of even chain sphingoid bases remains normal
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• methylmalonic acid levels are elevated in tissues and dried blood
• however, mice show normal ammonia levels
• mice fed a HP or PE diet show further elevations in methylmalonic acid levels
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• mice show an up-regulation in Lcn2 (lipocalin-2) at the mRNA and protein level in brain tissue, as well as increased protein levels of lipocalin-2 in kidney tissue
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• methylmalonic acid levels in urine are constantly elevated
• mice fed a HP or PE diet show further elevations in methylmalonic acid levels
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nervous system
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• mice show increased levels of the biomarker lipocalin-2 in the brain
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• increase in brain weight at 1 month and 1 year of age
• however, brain histology is normal in the basal ganglia
• mice fed a HP or PE diet show increased brain weight (normalized to body weight)
• mice fed a high protein diet and treated with cobalamin show a slight protective effect, with no increase in brain weight, although increases in metabolite levels still occur
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renal/urinary system
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• methylmalonic acid levels in urine are constantly elevated
• mice fed a HP or PE diet show further elevations in methylmalonic acid levels
|
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• mice show increased levels of the biomarker lipocalin-2 in kidney tissue indicating kidney damage
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• mice exhibit disturbed excretion of several electrolytes in urine, indicating renal tubular dysfunction
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• mice produce less urine during adulthood than controls, when normalized for body weight
• however, water intake is not different from controls
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mortality/aging
| N |
• mice exhibit a normal life span
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency | DOID:0060740 |
OMIM:251000 |
J:237040 | |
