behavior/neurological
N |
• despite increased energy expenditure, locomotor activity is similar to wild-type mice
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• body weight-adjusted food intake is increased in males compared to wild-type controls
• on a high fat diet food intake normalized to body weight is increase by 30%
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• when given a choice of diets male mice eat more of a diet lacking branched-chain amino acids compared to littermate controls
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growth/size/body
• increase in percent lean mass in males compared to wild-type controls
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• starting around 6 weeks of age body weight in males is 10 - 15% lower than in wild-type controls
• prior to 6 weeks of age male homozygous mice grow at the same rate as their littermate controls
• females are also lighter than controls but the difference is less than in males
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• mice are totally protected from high fat diet-induced obesity despite increase food intake (normalized to body weight)
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• somewhat enlarged in males
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adipose tissue
• weight is reduced by 55% in males compared to wild-type controls
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• in males
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homeostasis/metabolism
• protein synthesis and degradation are elevated
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• despite decreased consumption of branched-chain amino acids, plasma concentrations of Leu, Ile, and Val are increased 14-, 21-, and 31-fold, respectively, in male mice
• plasma concentrations of Leu, Ile, and Val are increased 25-, 33-, and 37-fold, respectively, in female mice
• plasma levels of Asp and Ala are decreased and levels of Thr, Cit, and Arg are increased in both males and females
• concentrations of the branched chain alpha-keto acids KMV and KIV are decreased in female mice
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• 24 h urine 3-methyhistidine and urinary 3-methyhistidine/creatinine ratio are increased by 52% and 78% respectively
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• creatinine concentration is reduced
• however, total urinary creatinine output is not different from wild-type mice
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• decreased by 88% in males
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• plasma concentrations of resistin are decreased by 34% in males
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• rectal core temperature is increased by 0.7oC postprandially (at 9 PM)
• however, rectal core temperature is similar to wild-type at 2 PM
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• VO2 normalize to body weight and on a whole mouse basis is increased
• respiratory quotient is increased during the initial (light) phase of a fast and during refeeding, but is similar to wild-type mice during longer fasting (dark phase)
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• mice are totally protected from high fat diet-induced obesity despite increase food intake (normalized to body weight)
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• fasting blood glucose is reduced by 31%
• however, fed glucose levels are not different from wild-type mice
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• fasting plasma insulin levels are reduced by 65%
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• in a glucose tolerance test blood glucose is significantly lower and the area under the curve is reduced by 51% compared to wild-type mice
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• plasma concentrations are decreased by 55% in males
• plasma concentrations are reduced by half in females
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• fasting plasma concentrations of beta-hydroxybutyrate are 50% lower compared to wild-type mice
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• fasting plasma concentrations are reduced by 40% compared to wild-type mice
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renal/urinary system
• somewhat enlarged in males
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• 24 h urine 3-methyhistidine and urinary 3-methyhistidine/creatinine ratio are increased by 52% and 78% respectively
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• creatinine concentration is reduced
• however, total urinary creatinine output is not different from wild-type mice
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