123 research outputs found
DDGS Based 3D Printing Biocomposite Filament
Additive manufacturing, also known as 3D printing, is a rapidly evolving technology with applications in many different industries. The market for 3D printing material, filament, is expected to grow to 325 million in revenue for North Dakota.North Dakota Corn Counci
Unraveling the Genetics of Human Obesity
The use of modern molecular biology tools in deciphering the perturbed biochemistry and physiology underlying the obese state has proven invaluable. Identifying the hypothalamic leptin/melanocortin pathway as critical in many cases of monogenic obesity has permitted targeted, hypothesis-driven experiments to be performed, and has implicated new candidates as causative for previously uncharacterized clinical cases of obesity. Meanwhile, the effects of mutations in the melanocortin-4 receptor gene, for which the obese phenotype varies in the degree of severity among individuals, are now thought to be influenced by one's environmental surroundings. Molecular approaches have revealed that syndromes (Prader-Willi and Bardet-Biedl) previously assumed to be controlled by a single gene are, conversely, regulated by multiple elements. Finally, the application of comprehensive profiling technologies coupled with creative statistical analyses has revealed that interactions between genetic and environmental factors are responsible for the common obesity currently challenging many Westernized societies. As such, an improved understanding of the different βtypesβ of obesity not only permits the development of potential therapies, but also proposes novel and often unexpected directions in deciphering the dysfunctional state of obesity
Genetic characterization of Italian patients with Bardet-Biedl syndrome and correlation to ocular, renal and audio-vestibular phenotype: identification of eleven novel pathogenic sequence variants
Ciliopathy is differentially distributed in the brain of a Bardet-Biedl syndrome mouse model
Bardet-Biedl syndrome (BBS) is a genetically heterogeneous inherited human disorder displaying a pleotropic phenotype. Many of the symptoms characterized in the human disease have been reproduced in animal models carrying deletions or knock-in mutations of genes causal for the disorder. Thinning of the cerebral cortex, enlargement of the lateral and third ventricles, and structural changes in cilia are among the pathologies documented in these animal models. Ciliopathy is of particular interest in light of recent studies that have implicated primary neuronal cilia (PNC) in neuronal signal transduction. In the present investigation, we tested the hypothesis that areas of the brain responsible for learning and memory formation would differentially exhibit PNC abnormalities in animals carrying a deletion of the Bbs4 gene (Bbs4-/-). Immunohistochemical localization of adenylyl cyclase-III (ACIII), a marker restricted to PNC, revealed dramatic alterations in PNC morphology and a statistically significant reduction in number of immunopositive cilia in the hippocampus and amygdala of Bbs4-/- mice compared to wild type (WT) littermates. Western blot analysis confirmed the decrease of ACIII levels in the hippocampus and amygdala of Bbs4-/- mice, and electron microscopy demonstrated pathological alterations of PNC in the hippocampus and amygdala. Importantly, no neuronal loss was found within the subregions of amygdala and hippocampus sampled in Bbs4-/- mice and there were no statistically significant alterations of ACIII immunopositive cilia in other areas of the brain not known to contribute to the BBS phenotype. Considered with data documenting a role of cilia in signal transduction these findings support the conclusion that alterations in cilia structure or neurochemical phenotypes may contribute to the cognitive deficits observed in the Bbs4-/- mouse mode. Β© 2014 Agassandian et al
Mutations in a Guanylate Cyclase GCY-35/GCY-36 Modify Bardet-Biedl SyndromeβAssociated Phenotypes in Caenorhabditis elegans
Ciliopathies are pleiotropic and genetically heterogeneous disorders caused by defective development and function of the primary cilium. Bardet-Biedl syndrome (BBS) proteins localize to the base of cilia and undergo intraflagellar transport, and the loss of their functions leads to a multisystemic ciliopathy. Here we report the identification of mutations in guanylate cyclases (GCYs) as modifiers of Caenorhabditis elegans bbs endophenotypes. The loss of GCY-35 or GCY-36 results in suppression of the small body size, developmental delay, and exploration defects exhibited by multiple bbs mutants. Moreover, an effector of cGMP signalling, a cGMP-dependent protein kinase, EGL-4, also modifies bbs mutant defects. We propose that a misregulation of cGMP signalling, which underlies developmental and some behavioural defects of C. elegans bbs mutants, may also contribute to some BBS features in other organisms
Hyperactive Neuroendocrine Secretion Causes Size, Feeding, and Metabolic Defects of C. elegans Bardet-Biedl Syndrome Mutants
Bardet-Biedl syndrome, BBS, is a rare autosomal recessive disorder with clinical presentations including polydactyly, retinopathy, hyperphagia, obesity, short stature, cognitive impairment, and developmental delays. Disruptions of BBS proteins in a variety of organisms impair cilia formation and function and the multi-organ defects of BBS have been attributed to deficiencies in various cilia-associated signaling pathways. In C. elegans, bbs genes are expressed exclusively in the sixty ciliated sensory neurons of these animals and bbs mutants exhibit sensory defects as well as body size, feeding, and metabolic abnormalities. Here we show that in contrast to many other cilia-defective mutants, C. elegans bbs mutants exhibit increased release of dense-core vesicles and organism-wide phenotypes associated with enhanced activities of insulin, neuropeptide, and biogenic amine signaling pathways. We show that the altered body size, feeding, and metabolic abnormalities of bbs mutants can be corrected to wild-type levels by abrogating the enhanced secretion of dense-core vesicles without concomitant correction of ciliary defects. These findings expand the role of BBS proteins to the regulation of dense-core-vesicle exocytosis and suggest that some features of Bardet-Biedl Syndrome may be caused by excessive neuroendocrine secretion
Human genetics and clinical aspects of neurodevelopmental disorders
This chapter traverses contemporary understandings of the genetic architecture of human disease, and explores the clinical implications of the current state of knowledge. Many different classes of genetic mutations have been implicated as being involved in predisposition to certain diseases, and researchers are continually uncovering other means by which genetics plays an important role in human disease, such as with somatic genetic mosaicism. Putative βde novoβ mutations can represent cases of parental mosaicism (including in the germline), which could be revealed by careful genotyping of parental tissues other than peripheral blood lymphocytes. There is an increasingly rich literature regarding rare mutations with seemingly large phenotypic effects. Privacy concerns have added to the difficulties of implementing genomics-guided medicine. With the advent of exome and whole genome sequencing (WGS), one needs to focus again on families over several generations, so as to attempt to minimize genetic differences, locus heterogeneity and environmental influences
Inventory Management by Selected Retail Farm Supply Co-ops -- Area III -- Wisconsin, Minnesota, North Dakota, South Dakota, and Northern Iowa
Excerpt from the report: This study was the third in a series on inventory management in farm supply cooperatives conducted by Farmer Cooperative Service. The purpose of this one was to: 1. Determine purchasing policies and practices that affect inventory acquisition. 2. Determine successful practices for storing and handling inventories. 3. Determine sales policies and practices which affect inventory turnover. 4. Recommend useful policies and practices for efficient inventory management
Evaluation of Pesticide Supplies and Demand For 1980
Pesticide supplies for the 1980 crop season should be adequate to meet overall farm needs. Net supplies should be about 2 percent greater than last year, and prices about 10 percent higher. Manufacturers' insecticide supplies are off by 3 percent, but there are larger, than normal stock carryovers for distributors, dealers, and farmers. Farmersβ herbicide requirements will rise about 2 to 5 percent this year. Insecticide use is likely to be up 8 to 10 percent over 1979βs abnormally low levels
Inventory Management by Selected Retail Farm Supply Co-ops - Area IV - Kansas, Nebraska, Missouri, Iowa, and Illinois
Excerpts from the report: This study was undertaken to determine inventory management policies and practices of farm supply cooperatives and their effect on operating efficiency. It is the fourth in a series of area studies on this subject conducted by Farmer Cooperative Service. Specifically the objectives of this study were to: 1. Determine purchasing policies and practices affecting inventory acquisition. 2. Determine successful handling and storage practices. 3. Determine sales policies and practices affecting inventories. 4. Recommend useful policies and practices for efficient inventory management. Representatives of Farmer Cooperative Service interviewed 23 local farm supply cooperatives to determine their inventory policies and practices and to obtain operating data pertaining to inventory management
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