Neuronal ceroid lipofuscinosis: clinical and morphologic findings in nine affected Polish Owczarek Nizinny (PON) dogs

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.     

PREDICTIVE INTELLIGENT CONTROL OF THE COLD STORAGE

The predictive intelligent control scheme is proposed, which is based on approximation theory and numerical method of Chcbyshev orthogonal polynomial. It has the practical value to the cold storge using widely electromagnetic valve and can be also applied similar systems in orther area.     

Land Storage System: Effect, Problem and Its Legal Regulation

The authors suggest that the land storage system is beneficial not only to strengthen the country's macroscopic regulation of city land market, accelerate healthy development of city economy and increase the financial income, it can also be advantageous to carry out the economic reform and public facilities construction without a hitch. On the basis of this, in this paper, the increment of the land price and house price as well as the high risk of fund due to lack of related laws is analyzed. Corresponding legal regulations, such as polishing the legislation of land right, implementing the land utilization planning, reinforcing the legislation of city development, land requisition and countryside residential land administration are put forward.     

A STUDY ON HEAT AND MOISTURE STATE OF HULL LAYER FOR THERMAL INSTULATION OF A COLD STORAGE

This paper analyses the thermal moist characteristics of the hull layer beside an air layer at lower temperature in an envelope for a cold storage and presents a new method of waterproof by air layer. With test data from engineering practice, the theory on the barrier of self moisture proof at the surface sublayer of the hull layer in an attic on a cold storage is proven. The dried zone about three phase point is confirmed. It is a new knowledge for thermophysics on thermal design of a cold storage with an optimum thermal performance.     

以粮温实测数据为例探讨低温储粮的可行性

通过对高大平房仓粮温进行跟踪检测，分析比较了粮温变化情况，并探讨了低温储粮的可行性。     

温室地下贮热系统设计研究

为了降低温室环境调控能耗。根据育苗不同阶段对气温与土壤温度的要求，设计了温室地下贮热系统，并阐述了系统的组成和贮热原理；同时，分析了各设计参数对系统贮热性能的影响。这样不仅为系统设计与理论分析奠定了基础，对其它类似地下贮热系统的设计、研究也具有参考意义。     

柑桔鲜果商品化处理对果品贮藏品质的影响

采用不同的商品化处理工艺流程，经过XLF-2型柑桔鲜果处理机组进行柑桔商品化处理及贮藏试验，对其品质进行了分析得出：鲜果经杀菌处理后不宜再经清水清洗，若是经过了清水清洗的果实，在涂蜡时必须加入杀菌保鲜剂。经商品化处理后的鲜果，色泽亮丽，品质佳，保鲜效果好，明显增加了其商品性，并可延长其货架期，但不宜长时间贮藏。     

干旱地区家庭雨水集储装置的设计

宁夏甘肃两省是我国严重缺水地区。缺水严重影响了该地区人民正常生活和工农业的发展。为此，针对家庭用水设计了屋顶雨水集储装置；在尽量减少建筑费用的情况下，选用了地下建池、拦污栅等污水处理装置，可为干旱地区建造家庭雨水集储装置提供一些参考依据。