Analysis Tools
growth/size/body
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• mice weigh 41% and 58% less than controls at 3 months and 9 months of age, respectively; weight loss is not due to selective muscle atrophy
• placing mice on a proline-free diet at 4 weeks of age worsens the weight loss phenotype
• proline present in standard chow is able to partially suppress the weight loss phenotype, and mice fed DietGel 76 A with or without added proline show near normal weight gain
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behavior/neurological
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• starting at 3 months of age, mice exhibit clasping of hindlimbs and forelimbs when suspended by the tail
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• at 3 months of age, grip strength is decreased by 33% for both the forelimbs and hindlimbs
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• at 3 months of age, mice show a reduction of 12% and 14% of lateral and longitudinal displacement, respectively, on the rear paws
• mice tend to keep their paws close to their body when walking, an effect not entirely explained by decreased body weight
• however, stance and swing times for both front and rear paws and stride length are normal
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adipose tissue
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• at 3 months of age, the thickness of the hypodermal fat layer is significantly reduced, regardless of the stage of the hair cycle
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• mice exhibit a 53% loss of total body fat relative to wild-type controls
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integument
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• at 3 months of age, the thickness of the hypodermal fat layer is significantly reduced, regardless of the stage of the hair cycle
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• at 3 months of age, skin is thin but not easily stretched
• collagen and elastin quantity and organization in the dorsal skin dermis are normal
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nervous system
| N |
• mice exhibit normal gross brain structure and a normal peripheral neuromuscular system; despite limb clasping, only a mild axonal atrophy is noted in peripheral nerves, with no axonal loss or functional defects
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• at 3 months of age, mice exhibit a slight but significant shift in the distribution of G-ratios in the motor branch of the femoral nerve, as shown by a population of smaller atrophic axons with proportionally thicker myelin
• however, the distribution of G-ratios is normal in the sciatic nerve and no defects in nerve conduction velocities or in the distance between nodes of Ranvier are noted in sciatic nerve at 3 or at 9 months of age
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• at 3 months of age, a population of smaller atrophic axons with proportionally increased myelin thickness is noted in the motor branch of the femoral nerve
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skeleton
| N |
• mice do NOT exhibit chronic hip dysplasia; no bone or joint abnormalities are observed
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• mice exhibit kyphosis at 9 months, but not at 3 months, of age; the angles of spine curvature are reduced to 55% and 80% of control values at the cervical and thoracic regions, respectively
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homeostasis/metabolism
| N |
• proline levels are normal both in serum and in lysates of primary skin fibroblasts
• in cell lysates, ornithine is not detected while glutamate, proline, and pyrroline-5-carboxylate (P5C) -- major precursors and intermediates in the proline synthesis pathway -- are similar to those in controls
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• at 3 months of age, mice show a 32% reduction in serum glutamate levels
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• blood urea nitrogen (BUN) level is significantly increased at 9 months of age
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• cholesterol are significantly decreased at 9 months, but not at 3 months, of age
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• serum HDL levels are significantly decreased at 9 months, but not at 3 months, of age
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• serum triglyceride levels are significantly decreased at 9 months, but not at 3 months, of age
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• serum potassium levels are low at 9 months, but not at 3 months, of age
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hematopoietic system
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• number of white blood cells is reduced at 9 months, but not at 3 months, of age
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• basophil number is significantly reduced at 9 months, but not at 3 months, of age
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• lymphocyte number is significantly reduced at 9 months, but not at 3 months, of age
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immune system
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• number of white blood cells is reduced at 9 months, but not at 3 months, of age
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• basophil number is significantly reduced at 9 months, but not at 3 months, of age
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• lymphocyte number is significantly reduced at 9 months, but not at 3 months, of age
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renal/urinary system
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• at 4 months of age, 50% of mice show fat droplets in the renal glomeruli
• however, no microalbuminuria is detected and urine levels of sodium and potassium are normal
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