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Neurological Manifestations of Niemann-Pick Disease Type C in Cats   总被引:3,自引:1,他引:2  
Seven Domestic shorthair cats with a lysosomal storage disorder analogous to human Niemann-Pick disease type C, from a breeding colony were studied to characterize the neurological manifestations of this disorder. Affected cats were identified by means of liver biopsies at 4 to 6 weeks of age. Neurological examinations were performed at 2 week intervals from the onset of clinical signs. All cats displayed signs referrable to the cerebellum, with a subtle intention tremor noticed initially at 8 to 12 weeks of age; the disease was rapidly progressive. The tremor became more pronounced, menace response was lost, and severe dysmetria and ataxia developed. Three cats also had signs referrable to other areas of the central nervous system. Cats died or were euthanized between 12 and 43 weeks of age. Pathological findings included accumulation of substrate within neurons throughout the central nervous system, and axonal spheroid formation. The clinical and pathological findings in these cats are comparable to those in the human form of the disease.  相似文献   
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The objective of this study was to determine if acute and chronic changes in circulating metabolic hormone and metabolite concentrations are associated with β-agonist-induced nutrient repartitioning in young growing lambs. Two groups of 12 Dorset and Dorset-Finn cross ram lambs weighing 36 or 33 kg live weight were assigned to 3- or 6-week treatment intervals, respectively, to achieve similar slaughter weights. Six lambs within each treatment interval were fed ad libitum a complete mixed high-concentrate diet containing either 0 or 10 ppm cimaterol. During the first 12 hr of cimaterol administration plasma somatotropin (ST), thyroxine (T4), and triiodothyronine (T3) concentrations were not altered by treatment, but plasma insulin, glucose, non-esterified fatty acids (NEFA) and glycerol concentrations were elevated 2 hr after ingestion. These acute responses suggest direct stimulation of glycogenolysis and lipolysis by cimaterol, which is characteristic of β-adrenergic alteration of carbohydrate and lipid metabolism. Chronic administration of cimaterol significantly decreased insulin concentrations by 36% and 52% at 3 and 6 weeks, respectively, while glucose concentrations remained unchanged. Serum IGF-I concentrations were not significantly altered by cimaterol. T4 levels were reduced 22.1% after 3 weeks of cimaterol treatment. Although plasma NEFA concentrations were chronically elevated 56% to 65% in lambs fed cimaterol, plasma glycerol concentrations remained at baseline levels. The relative changes in plasma NEFA and glycerol concentrations are consistent with a decreased rate of lipogenesis, rather than an increase in lipolysis.  相似文献   
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The effects of bovine growth hormone (GH) and thyroxine (T4) on growth and carcass characteristics were assessed in Dorset ram lambs. Lambs in four groups (n = 10/group) were treated for 30 d as follows: controls, 3.33 mg (6 IU) GH/d (s.c.); 5-mg T4 implant (s.c.) on d 1 and a 10-mg T4 implant 21 d later; GH + T4. Blood samples were collected at 3-d intervals for analysis of GH, T4, triiodothyronine, somatomedin-C and testosterone concentrations. Six lambs/group were slaughtered for carcass measurements and composition. Daily GH injections increased (P less than .005) baseline plasma GH levels 10-fold, whereas plasma T4 concentrations were increased 10% (P less than .10) by the implants. Somatomedin-C increased with time in all groups, but the increments from d 0 to d 30 were higher (P less than .05) with GH treatment. Average daily gain (mean = 352 g/d), feed consumption and feed to gain ratio were not affected (P greater than .1) by GH or T4 treatment in ram lambs. Hot carcass weight and dressing percentage were increased (P less than .05) by T4. Growth hormone increased carcass protein content (P less than .005) and muscle weights while reducing carcass fat (P less than .05). Carcass composition was not altered by T4 alone, and the T4 x GH interaction was not significant; however, the combination of T4 and GH resulted in greater muscle and protein weight than did either hormone alone or no hormone administration. There were no differences in bone length or in the metacarpal growth plate width among groups. The beneficial effects of GH on carcass composition were not further enhanced by administration of thyroxine.  相似文献   
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Rainbow trout Oncorhynchus mykiss (261.6 × 24.7 g initial weight, mean × SEM) at 13.1 × 0.2 C were exposed for 94 d to one of three CO2 treatments: control (22.1 × 2.8 mg/L), medium (34.5 × 3.8 mg/L), or high (48.7 × 4.4 mg/L). Trout were checked daily for survival, and fish were sampled at 0, 28, 56, and 84 d for physiological responses, growth, and fillet quality assessments. Trout were also challenged to a 15-min crowding stress at 93 d to assess their ability to initiate a stress response during hypercapnia. Chronically exposed trout showed nearly 100% survival through 84 d exposure (1 of 1,500 fish died). Growth and physiological results showed that increasing elevated CO2, concentrations result in corresponding decreased growth rates and CO2specific physiological parameters: The medium and high CO2 treatments had significantly slower growth and subsequently smaller fish by 84 d. Exposed trout also showed significantly ( P < 0.05) decreased plasma chloride for medium and high CO2 treatments compared to the control from 28 through 84 d. Decreased growth and smaller fish in the medium and high CO2 treatments resulted in correspondingly smaller fresh and smoked fillet weights. Chronic CO2 exposure did not result in notable changes in ultimate muscle pH. Exposure to 15-min crowding stress at 93 d resulted in significant changes in hematocrit, plasma cortisoI, glucose, and chloride for all treatment groups. CO2-specific changes were detected in hematocrit, plasma cortisoI, and plasma chloride responses following the 15-min crowding stress.  相似文献   
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