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Australian Cattle Dogs with Neuronal Ceroid Lipofuscinosis are Homozygous for a CLN5 Nonsense Mutation Previously Identified in Border Collies 下载免费PDF全文
A. Kolicheski G.S. Johnson D.P. O'Brien T. Mhlanga‐Mutangadura D. Gilliam J. Guo T.D. Anderson‐Sieg R.D. Schnabel J.F. Taylor A. Lebowitz B. Swanson D. Hicks Z.E. Niman F.A. Wininger M.C. Carpentier M.L. Katz 《Journal of veterinary internal medicine / American College of Veterinary Internal Medicine》2016,30(4):1149-1158
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Staniar WB Kronfeld DS Akers RM Harris PA 《Journal of animal physiology and animal nutrition》2007,91(9-10):390-399
The objective of this longitudinal study was to characterize growth and plasma insulin-like growth factor I (IGF-I) concentrations in pasture-raised thoroughbreds fed two sources of dietary energy. Mares and foals were randomly assigned to either a sugar and starch (SS) or fat and fibre (FF)-rich feed, and plasma IGF-I and growth were measured once a month from 1 to 16 months of age. These dependent variables were also compared with day length and ambient temperature. There was an association between plasma IGF-I concentration and average daily gain (ADG) (r = 0.32, p < 0.001). There were also clear seasonal patterns in both ADG and plasma IGF-I, with high values in June and May, and a low value in March. Plasma IGF-I and ADG were positively associated with day length and temperature. Plasma IGF-I was never higher (p > 0.10) in the FF group when compared with the SS group, and was higher in the SS group during a rapid growth phase in the spring of year 2 (p < 0.10). The results establish an association between ADG and IGF-I in the horse and indicate that environment and age may influence this relationship. In addition, plasma IGF-I is influenced by dietary energy source at particular times of year. This link has important implications in designing feeding management strategies that are aimed at addressing skeletal development. 相似文献
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OBJECTIVE: The aim of this study was to characterize the clinical and morphologic features of neuronal ceroid lipofuscinosis (NCL) in the Polish Owczarek Nizinny (PON) breed of dog. ANIMALS: Nine Swedish PON dogs of both sexes were included in the study. PROCEDURE: All dogs underwent a detailed clinical evaluation, with emphasis on ophthalmic exams. Histopathology and electron microscopy were performed on the eyes, brain and various internal organs. Immunohistochemical staining for detection of sphingolipid activator proteins (SAPs) and mitochondrial ATP synthase (SCMAS) was performed on the eyes and brain. RESULTS: The dogs showed behavioral abnormalities, motor disturbances and visual impairment or blindness. Pupillary responses were abnormal while fundus changes varied from normal to severe retinal atrophy. Electroretinography (ERG) showed variable changes, from slight alterations in the process of dark adaptation to severely reduced or nonrecordable ERG a- and b-wave amplitudes. Histopathology revealed intracytoplasmic storage bodies within neurons of the brain and in retinal cells, especially the retinal pigment epithelium (RPE). Round to oval granular type of inclusion bodies, known as granular osmiophilic dense deposits (GRODS), were found in neuronal cells in the brain and in the retina. Immunohistochemistry identified the storage material in the brain and retina as consisting of SAPs. CONCLUSION: The presently described NCL disease in PON dogs shows similarities to previously recorded cases in the Miniature Schnauzer. The closest human equivalent to this disease is infantile NCL (CLN1), in which the major stored proteins are SAPs and the ultrastructure of the inclusion bodies of neuronal cells is granular. 相似文献
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试验表明,三道湾水电站原设计存在以下问题:泄洪排沙洞单独全开运行工况下,其进口上方存在贯通性吸气漏斗漩涡;正常溢洪洞进口侧收缩十分严重,泄流量不足,闸后存在水翅、折冲波等不良流态,下游消能效果不佳;针对实际情况,试验通过在泄洪排沙洞进口增设消涡格栅,在正常溢洪洞进口设置椭圆形导墙、延长闸后收缩段,下游采用扩散挑流消能等措施,很好地解决了上述问题。 相似文献
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