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Caprine beta-mannosidosis is an autosomal recessive defect of glycoprotein catabolism with a deficiency of tissue and plasma beta-mannosidase activity and tissue accumulation of oligosaccharides within lysosomes. This rapidly fatal genetic disorder of Nubian goats is expressed at birth by a variety of clinical signs including deafness. Affected goats had folded pinnas, and the tympanic cavity was decreased due to multiple, polypoid projections of bone covered by middle ear mucosa which obstructed the view of the cochlear promontory. Numerous cells of the cochlear duct including mesothelial and epithelial cells of Reissner's membrane, mesothelial cells lining the scala tympani, cells of the stria vascularis, numerous supportive cells of the organ of Corti, cochlear hair cells, endothelial cells, perithelial cells, fibroblasts, macrophages, and neurons of the spiral ganglion contained numerous nonstaining intracytoplasmic vacuoles which resulted in distention of affected cells and caused thickening of involved structures. Ultrastructurally, the vacuoles were membrane-bound and consistent with lysosomes. Vacuolated cells were desquamated into the scala vestibuli and scala tympani. This is one of few reports describing light and electron microscopic otic alterations of a storage disease. Goats with beta-mannosidosis appear to be good models of hearing loss in patients with storage disease.  相似文献   

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The clinical features of caprine beta-mannosidosis were evaluated in 10 newborn goats, one stillborn goat and one goat fetus. The phenotypic abnormalities observed in all 10 live affected animals included an inability to rise from a recumbent position, moderate to marked intention tremor, eye movements resembling pendular nystagmus, clinical deafness, bilateral Horner's syndrome, carpal contractures, pastern joint hyperextension, thickened skin and to a varying degree, a dome-shaped skull. Subjective evaluation suggested that most animals had a decreased muscle mass. Together, these characteristics represent a common phenotype which is expressed at birth in caprine beta-mannosidosis.  相似文献   

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Fifty dairy goats, of various ages, sexes and breeds were selected for examination on the basis of positive serological reactions to caprine arthritis-encephalitis virus (CAEV). Thirty-one had lung lesions including chronic interstitial pneumonia of caudal or cranioventral lobes, bronchopneumonia, verminous pneumonia, pulmonary cryptococcosis or combinations of these. The only infective agent recovered from all the chronic interstitial pneumonia cases examined was CAEV, which was also recovered from lung tissue of 3 goats with arthritis but no lung lesions. The presence of CAEV in lavaged alveolar macrophages from normal lung tissue and from lungs affected with chronic interstitial pneumonia and verminous pneumonia, and the demonstration of a marked increase in nonspecific esterase staining macrophages in areas of chronic interstitial pneumonia, are discussed in relation to the aetiology of the pneumonia.  相似文献   

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Goats that had been inoculated with the causal organism of contagious, caprine pleuropneumonia and treated, within a few days, with oxytetracycline or tylosin, were less severely affected than infected, untreated control goats. However, 20% of treated cases remained infective and were, presumably, capable of transmitting the infection.  相似文献   

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应用RT-PCR方法从山羊外周血淋巴细胞中克隆了山羊白细胞介素-2(Caprine interleukin-2,CapIL-2)基因并进行测序,将编码山羊IL-2蛋白的基因亚克隆至pET30a( )上,转化大肠杆菌BL21(DE3)并用IPTG于37℃诱导培养。表达融合蛋白通过ProBondTM蛋白纯化试剂盒纯化。纯化产物经3次免疫新西兰白兔,制备出山羊IL-2抗血清。本次试验成功地表达、纯化了山羊IL-2,并制备出兔抗山羊IL-2抗血清,为下一步开展山羊IL-2重组蛋白的应用研究奠定了基础。  相似文献   

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Plasma beta-mannosidase activities were determined for Salers cattle from 8 herds as an evaluation of this method for detection of beta-mannosidosis heterozygotes. Several biological factors, such as age, gender, herd, and risk of being a beta-mannosidosis carrier, were considered in this study. The mean enzyme activity for obligate heterozygotes (n = 8) was 55 U/ml (range = 43-65 U/ml), which was 59% of the mean enzyme activity for cattle that were low risk for being a carrier. These data indicate that bovine beta-mannosidosis is characterized by a gene dosage effect. The analytical and biological variation of plasma beta-mannosidase activity that was observed necessitates limiting the test to adult fullblood/purebred Salers cattle within a herd. Plasma beta-mannosidase analysis provides important information for intraherd selection of Salers cattle that are heterozygous for beta-mannosidosis.  相似文献   

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In a field disease outbreak, 60 female goats died over a 2-3 week period. Necropsies of seven of these does revealed that six had an acute exudative necrotising broncho-pneumonia, and moderate to high numbers of Pasteurella haemolytica were isolated from their lungs. Caprine herpesvirus, identified as Bovid herpesvirus type 6, was isolated from the lungs of two of these does, including one with pneumonia, and from nasal swabs of in-contact goats. Clinical disease was only observed in does, and deaths began 3 weeks after the introduction of a mob of goat hoggets from another farm.  相似文献   

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山羊关节炎-脑炎(Caprine arthritis encephalitis,CAE)是由反转录病毒科慢病毒属山羊关节炎脑炎病毒(CAEV)引起的山羊的慢性传染病。临床症状以山羊羔脑脊髓炎和成年山羊多发性关节炎、硬结性乳房炎以及间质性肺炎为特征。在未认识该病之前,对其说法不一。1964年Stunzi在瑞士称其为山羊慢性淋巴细胞性多发性关节炎;1969年Starrou在德国发现,称其为山羊肉芽肿性脑脊髓炎;1974年Cark等在美国报道为山羊病毒性白质脑脊髓炎。  相似文献   

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The caprine oxyhemoglobin dissociation curve has not been previously defined. Blood from 10 healthy goats was equilibrated in a tonometer with calibrated gas mixtures of oxygen at concentrations of 95%, 21%, 13%, 12%, 10%, 9%, 8%, 5%, 4%, and 2.5% , 5% carbon dioxide, balance nitrogen. The pH, partial pressure of oxygen (PO2), partial pressure of carbon dioxide (PCO2), total hemoglobin, oxyhemoglobin saturation, carboxyhemoglobin, methemoglobin, and oxygen content were measured. The PO2/oxyhemoglobin and the PO2/oxygen content relationships were graphed with curve-fitting software and a formula for calculating oxyhemoglobin from PO2 was generated. The maximum oxygen content per gram of hemoglobin was 1.29 ml of oxygen per gram of hemoglobin. The PO2 at which hemoglobin was 50% saturated (P50) from the PO2/oxyhemoglobin relationship was 28.6+/-1.5 mmHg and that from the PO2/oxygen content relationships was 29.1+/-1.6 mmHg. The Hill coefficient for the PO2/oxyhemoglobin data was 3.0+/-0.4.  相似文献   

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Pathogenic mechanisms of caprine arthritis-encephalitis virus   总被引:1,自引:0,他引:1  
Goats infected with caprine arthritis-encephalitis virus (CAEV) show chronic arthritis and cachexia, which are progressive in nature. The immunopathogenic mechanisms responsible for these progressive clinical symptoms have not been fully elucidated. Various haematological and immunological parameters were evaluated in experimentally-infected goats showing typical signs of CAEV-induced disease. Infected goats showed recurrent lymphocytosis that may be due to constant presentation of antigen by infected cells of a monocyte/macrophage lineage. The serum alkaline phosphatase and -glutamyl transferase concentrations were elevated in infected goats, a characteristic of hepatic and bone disorders. All other serum chemistry parameters were similar between infected and control goats. Importantly, the serum tumour necrosis factor- (TNF-) levels were higher in infected goats. The cachexia seen in infected goats may be at least partly due to altered metabolism as a result of prolonged elevation of serum TNF- levels. Depressed natural killer cell activity was observed in infected goats and may contribute towards the establishment of a persistent infection with CAEV.Abbreviations AIDS acquired immunodeficiency syndrome - CAEV caprine arthritis-encephalitis virus - GGT -glutamyl transferase - HBSS Hanks' balanced salt solution - HIV human immunodeficiency virus - NK natural killer - PBMC peripheral blood mononuclear cells - PCR polymerase chain reaction - SAP serum alkaline phosphatase - TNF tumour necrosis factor  相似文献   

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