(39)), by contrast, Y2 receptors have not been detected on bone. In addition to effects in bone, Y1 receptors have been considered as important regulators of energy homeostasis, consistent with pharmacological evidence from Y receptor agonists and antagonists to stimulate or inhibit feeding (9). Fasting-induced re-feeding is reduced in germ line Y1 receptor knock-out mice (10), and deletion of Y1 receptors in genetically obese ob/ob mice, in which hypothalamic NPY-ergic activity is chronically increased, significantly reduces food intake and body weight (11). Paradoxically, germ line Y1 receptor knock-out mice develop late-onset obesity in the absence of hyperphagia (10,12,13). One hypothesis to reconcile this apparent discrepancy is that hypothalamic and non-hypothalamic Y1 receptors have different effects on energy homeostasis.Given the clear involvement of Y1 receptors in the regulation of energy homeostasis as well as new evidence of a putative role for Y1 receptors on osteoblast-like cells, we investigated the effect of germ line and conditional (adult-onset, hypothalamus-specific) deletion of Y1 receptors in mice. In addition, the potential interaction between Y1 receptor sig-
Over the past decade there have been major advances in defining the genetic basis of the majority of congenital myopathy subtypes. However the relationship between each congenital myopathy, defined on histological grounds, and the genetic cause is complex. Many of the congenital myopathies are due to mutations in more than one gene, and mutations in the same gene can cause different muscle pathologies. The International Standard of Care Committee for Congenital Myopathies performed a literature review and consulted a group of experts in the field to develop a summary of (1) the key features common to all forms of congenital myopathy and (2) the specific features that help to discriminate between the different genetic subtypes. The consensus statement was refined by two rounds of on-line survey, and a three-day workshop. This consensus statement provides guidelines to the physician assessing the infant or child with hypotonia and weakness. We summarise the clinical features that are most suggestive of a congenital myopathy, the major differential diagnoses and the features on clinical examination, investigations, muscle pathology and muscle imaging that are suggestive of a specific genetic diagnosis to assist in prioritisation of genetic testing of known genes. As next generation sequencing becomes increasingly used as a diagnostic tool in clinical practise, these guidelines will assist in determining which sequence variations are likely to be pathogenic.
Bone tissue engineering has emerged as one of the leading fields in tissue engineering and regenerative medicine. The success of bone tissue engineering relies on understanding the interplay between progenitor cells, regulatory signals, and the biomaterials/scaffolds used to deliver themotherwise known as the tissue engineering triad. This review will discuss the roles of these fundamental components with a specific focus on the interaction between cell behaviour and scaffold structural properties. In terms of scaffold architecture, recent work has shown that pore size can affect both cell attachment and cellular invasion. Moreover, different materials can exert different biomechanical forces, which can profoundly affect cellular differentiation and migration in a cell type specific manner. Understanding these interactions will be critical for enhancing the progress of bone tissue engineering towards clinical applications.
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