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111.
CASE HISTORY: A skeletal disease characterised by dwarfism, limb deformity and sometimes sudden death occurred over a period of 5 years in lambs born on a commercial sheep farm in Southland. The disease showed variable expression and occurred in crossbred sheep. A genetic aetiology was supported by the birth of affected lambs over two seasons in a flock of putative carrier and affected sheep transported to Massey University.

CLINICAL FINDINGS: Affected lambs appeared normal at birth but showed evidence of dwarfism, wide-based stance and exercise intolerance as early as 1 week of age. Most died within the first 3 months of life, often after developing bilateral varus deformity of the forelimbs. Some severely-affected lambs died suddenly of respiratory embarrassment, probably due to tracheal collapse. Mildly-affected individuals had a short, blocky stature and some survived to breeding age.

PATHOLOGICAL FINDINGS: Gross and microscopic lesions of variable severity were present in the tracheal, articular, epiphyseal and physeal cartilages. In severe cases, articular cartilage in major joints was eroded from weight-bearing surfaces. The trachea was flaccid, abnormally kinked, and had thickened cartilaginous rings and a narrow lumen. Affected sheep that survived to breeding age eventually developed severe degenerative joint disease. Histologically, chondrocytes were disorganised, surrounded by concentric rings of abnormal fibrillar material, and the matrix often contained focal to coalescing areas of chondrolysis.

DIAGNOSIS: Inherited chondrodysplasia of Texel sheep.

CLINICAL RELEVANCE AND CONCLUSIONS: This chondrodysplasia differs from those previously described in sheep and is considered to be a newly-recognised, recessively-inherited genetic disease of the Texel breed. A defect in the synthesis of glycosaminoglycans in cartilage matrix is suspected. This disease of sheep may provide a suitable model for studying various forms of therapy for human chondrodysplasias.  相似文献   
112.
Inherited rickets of Corriedale sheep is of simple autosomal recessive inheritance. The gross and histological lesions include segmental thickening of physes, persistence of hypertrophic chondrocytes at sites of endochondral ossification, excessive osteoclastic resorption, microfractures and wide unmineralised osteoid seams lining trabeculae and filling secondary osteons. Initial testing revealed concentrations of 25-hydroxyvitamin D3 were normal, but those of 1,25-dihydroxyvitamin D3 were increased in serum of affected sheep, suggesting a defect in the vitamin D receptor. However, in vitro studies on cultured skin fibroblasts revealed normal binding and function of the vitamin D receptor.

A genome-wide association study was conducted using the Illumina OvineSNP50 BeadChip. A homozygous region of 199 consecutive single-nucleotide polymorphism loci was identified in affected sheep, covering a region of 10 megabase pairs on ovine chromosome 6. Of the 91 candidate genes in this region, dentin matrix protein 1 gene (dmp1) was considered the most likely as it is known to play critical roles in osteocyte maturation and mineral metabolism. Sequencing of DNA from carriers showed a nonsense mutation 253T/C in dmp1. This T/C transition introduced a stop codon (R145X) that could truncate C-terminal amino acids. Using PCR restriction fragment length polymorphism for this mutation, genotyping showed that all affected sheep were “TT” genotypes, and phenotypically normal sheep were either “CT” or “CC”. These findings suggest that a mutation in dmp1 is responsible for inherited rickets of Corriedale sheep. A simple diagnostic test is currently being designed to identify carriers with the defective “T” alleles.  相似文献   
113.
Abstract

HISTORY: A similar episodic neurological disorder occurred in new born lambs on two unrelated properties involving disparate breeds of sheep. Because of the number of lambs born, cross-breeding and the fact it occurred in some mating groups and not others, a dominant mode of inheritance was, initially and separately, suspected in each case. The sires of affected lambs were apparently normal. Whereas one was New Zealand Romney, the other was a composite breed with East Friesian genetics, but both rams originated from the same source property. To investigate the pathogenesis of the disorder these two rams were acquired and mated with unrelated sheep, under experimental conditions in a more controlled environment.

CLINICAL FINDINGS: A proportion of lambs born to both sires exhibited a similar neurological disorder. Some lambs were noted to be abnormal at birth, both on home properties and in the experimental flock. They tended to adopt a head and neck extended posture and were slow to get to their feet and suckle when they then became more or less normal. When forced to move, they and other more robust lambs elicited an asymmetric gait, base-wide extensor hypertonia (hypometria) of thoracic limbs and flexor hypertonia (hypermetria) of pelvic limbs. In some there was nystagmus. After several metres of asymmetric ataxic gait they would fall to one side, sometimes adopting a sitting position. Recovery usually occurred in one to several minutes. As lambs aged, it became more difficult to elicit the episodes of dysfunction and by 6 months of age they appeared normal.

DIAGNOSIS: The disorder was diagnosed as a dominant familial episodic cerebellovestibular ataxia inherited as a dominant trait, with incomplete penetration of observed clinical signs and variable expressivity.

CLINICAL RELEVANCE: A proportion of affected lambs are likely to die in the neonatal period so the specific nature of the disorder may go unrecognised. Because of incomplete penetrance and varying expressivity, many of the lambs carrying this mutation will survive without showing clinical signs and may enter breeding flocks, where the disorder may be perpetuated and contribute to neonatal deaths.  相似文献   
114.
During the period of August 2002 and November 2004, an epidemiological investigation for Bartonella infection was conducted in small mammals in Taiwan. Using whole blood culture on chocolate agar plates, Bartonella species were successfully isolated from 41.3% of the 310 animals tested. The isolation rate of Bartonella species varied among different animal species, including 52.7% of the 169 Rattus norvegicus, 28.6% of the 126 Sucus murinus, 10% of the 10 Rattus rattus and 66.7% of the three Rattus losea. Bacteremia prevalence also varied with the origin of the animals, as 56.2% of the animals captured on farms, 38.6% of the ones captured at harbour sites and 11.8% of the animals captured from urban areas were bacteremic. Through molecular analysis of the gltA gene and 16S/23S intergenic spacer region, genetic diversity of Bartonella organisms was identified, including strains closely related to Bartonella tribocorum, Bartonella grahamii, Bartonella elizabethae, Bartonella phoceensis and Bartonella rattimassiliensis. Moreover, this is the first report of zoonotic B. elizabethae and B. grahamii identified in R. losea, the lesser rice‐field rat. Various Bartonella species were identified in R. norvegicus, compared to 97.2% of Suncus murinus with unique Bartonella species. By indirect immunofluorescence antibody test, using various rodent Bartonella species as antigens, consistently low percentage of seropositivity implied that small mammals may play a role as competent reservoirs of Bartonella species in Taiwan. Future studies need to be conducted to determine whether these Bartonella species would be responsible for human cases of unknown fever or febrile illness in Taiwan, especially zoonotic B. elizabethae and B. grahamii.  相似文献   
115.
Ehrlichia (E.) canis is a Gram-negative obligate intracellular bacterium responsible for canine monocytic ehrlichiosis. Currently, the genetic diversity of E. canis strains worldwide is poorly defined. In the present study, sequence analysis of the nearly full-length 16S rDNA (1,620 bp) and the complete coding region (4,269 bp) of the gp200 gene, which encodes the largest major immunoreactive protein in E. canis, from 17 Taiwanese samples was conducted. The resultant 16S rDNA sequences were found to be identical to each other and have very high homology (99.4~100%) with previously reported E. canis sequences. Additionally, phylogenetic analysis of gp200 demonstrated that the E. canis Taiwanese genotype was genetically distinct from other reported isolates obtained from the United States, Brazil, and Israel, and that it formed a separate clade. Remarkable variations unique to the Taiwanese genotype were found throughout the deduced amino acid sequence of gp200, including 15 substitutions occurring in two of five known species-specific epitopes. The gp200 amino acid sequences of the Taiwanese genotype bore 94.4~94.6 identities with those of the isolates from the United States and Brazil, and 93.7% homology with that of the Israeli isolate. Taken together, these results suggest that the Taiwanese genotype represents a novel strain of E. canis that has not yet been characterized.  相似文献   
116.
Although the proteins BAX and BAK are required for initiation of apoptosis at the mitochondria, how BAX and BAK are activated remains unsettled. We provide in vivo evidence demonstrating an essential role of the proteins BID, BIM, and PUMA in activating BAX and BAK. Bid, Bim, and Puma triple-knockout mice showed the same developmental defects that are associated with deficiency of Bax and Bak, including persistent interdigital webs and imperforate vaginas. Genetic deletion of Bid, Bim, and Puma prevented the homo-oligomerization of BAX and BAK, and thereby cytochrome c-mediated activation of caspases in response to diverse death signals in neurons and T lymphocytes, despite the presence of other BH3-only molecules. Thus, many forms of apoptosis require direct activation of BAX and BAK at the mitochondria by a member of the BID, BIM, or PUMA family of proteins.  相似文献   
117.
In the first stage, polyethylene terephthalate (PET) fibers and Kevlar fibers are combined at a blending ratio of 80/ 20 wt% in order to form PET/Kevlar nonwoven fabrics. Two pieces of PET/Kevlar nonwoven fabrics that enclose a carbonfiber (CF) interlayer are then needle punched in order to form PET/Kevlar/CF (PKC) composites. In the second stage, the sandwiches compose PKC composites as the top and the bottom layers, as well as an interlayer that is composed of a spacer fabric and polyurethane (PU) foam. PU foams have different densities of 200, 210, 220, 230, and 240 kg/m3. These resulting nonwoven fabric/spacer fabric/PU foam sandwiches are then tested using a drop-weight impact test, a compression test, a bursting strength test, a sound absorption test, and a horizontal burning test. The test results indicate that the optimal properties of sandwiches occur with their corresponding PU foam density as follows: an optimal residual stress (240 kg/m3), an optimal compressive strength (240 kg/m3), and an optimal bursting strength (220 kg/m3). In addition, the sandwiches reach the HF1 level according to the horizontal burning test results. They also have an average electromagnetic interference shielding effectiveness of -48 dB, as well as a sound absorption coefficient of 0.5 in a frequency between 1500-2500 Hz, which indicates a satisfactory sound absorption effect. The nonwoven fabric/spacer fabric/PU foam sandwiches proposed in this study are mechanically strong, sound absorbent, and fire retardant, and can be used in construction material and electromagnetic shielding composites.  相似文献   
118.
Synthetic CpG-oligodeoxynucleotides (CpG-ODN) are potent adjuvants that accelerate and boost antigen-specific immune responses. Toll-like receptor 9 (TLR9) is the cellular receptor for these CpG-ODN. Previous studies have shown species-specific activation of mouse TLR9 (mTLR9) and human TLR9 (hTLR9) by their optimized CpG-ODN. The interaction between rabbit TLR9 (rabTLR9) and CpG-ODN, however, has not been previously investigated. Here, we cloned and characterized rabTLR9 and comparatively investigated the activation of the rabbit, mouse, and human TLR9 by CpG-ODN. The complete open reading frame of rabTLR9 encodes 1028 amino acid residues, which share 70.6% and 75.5% of the identities of mTLR9 and hTLR9, respectively. Rabbit TLR9 is preferentially expressed in immune cells rich tissues, and is localized in intracellular vesicles. While mTLR9 and hTLR9 displayed species-specific recognition of their optimized CpG-ODN, rabbit TLR9 was activated by these CpG-ODN without any preference. This result suggests that rabTLR9 has a broader ligand-recognition profile than mouse and human TLR9.  相似文献   
119.
Most eucalypt plantations are managed in short rotations (6–8 years) and are established in regions with water and nutritional stresses of varying degrees. The mean annual increment in these regions ranges from 20 to 45 m3 ha?1 y?1 depending on the level of environmental stress. Improving natural resource use efficiency by breeding and matching genotypes to sites and using appropriate site management practices is a key challenge to sustain or increase productivity. Climate change is emerging as a driving force behind many of the current issues facing natural resources, and is likely to intensify the water constraint. The Intergovernmental Panel on Climate Change has predicted significant increases in surface air temperatures and decreases in rainfall (with more erratic rainfall patterns) over the tropics and subtropics, affecting drier regions in particular. To be truly sustainable, forest management practices must be environmentally friendly, prevent or reduce land degradation, improve biodiversity and increase resilience to climate variation and change. The main efforts should address the problems of water scarcity, low soil fertility and reduced biodiversity. Forest growers should take into account the inherent risks of different forest management systems on the regional water availability and consider reducing forest productivity in order to maintain both ecological and social equilibrium. The design and implementation of management techniques should be adapted to the specific production and protection objectives, which depend on the regional and local edaphoclimatic circumstances, water resources management, protection against soil erosion, diseases, pests, bushfires, strong winds and alien species. This paper addresses the basic requirements for integrating genetic and silvicultural strategies to minimise the water constraints in eucalypt plantations and surrounding water and native vegetation resources. We show several examples of effective forest management actions on the scale of forest stand and watershed in use in Brazil.  相似文献   
120.
The analysis of salbutamol in swine serum is the more practical basis for large scale surveillance programs in Taiwan. Objectives of the study were to develop a new assay and to compare with a commercially available kit in field test screens. A simple and reliable enzyme-linked immunosorbent assay (ELISA) to monitor the presence of beta-agonist, salbutamol, in 1,358 field samples of swine serum that were collected from local meat markets was described. The method proved to be suitable and sensitive for the detection of beta-agonist residues caused by growth promoting dosage. The limit of detection of the developed ELISA directly performed on diluted serum was 0.25 ng/mL. The application and the results of two ELISA kits (homemade and commercially available) for the screening of salbutamol were presented. For further confirmation, all samples that showed to be ELISA positive for salbutamol residues were analyzed by GC-MS. Adopting 1 ng/mL salbutamol as a cutoff value, the commercial beta-agonist ELISA had a sensitivity of 89.2% and a specificity of 86.7% versus GC-MS at a cutoff of 1 ng/mL. The homemade salbutamol ELISA had a sensitivity of 81.1% and a specificity of 98.6% and gave a low proportion of false-positive rate results. The reliability of the developed kit in terms of the percentage of false-positive rate results is evaluated. In conclusion, a sensitive, specific salbutamol ELISA has been developed that could serve as a rapid screening assay, and the detection of positive samples at the place of sampling can result in more effective control of the illegal use of beta-agonists.  相似文献   
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