Claire Bovet, Jennifer Stevens-Lapsley, and Matthew RG Taylor*
Introduction: Late-onset Pompe disease is a rare glycogen storage disorder characterized by skeletal myopathy and respiratory failure. Enzyme replacement therapy using recombinant alglucosidase alfa improves functional and respiratory status across cohorts, but quantitative measurements of an individual’s response to enzyme replacement therapy are poorly characterized. Here we report quantitative improvements of muscle strength and functional performance in a patient with late-onset Pompe disease.
Methods: A 23-year-old patient with late-onset Pompe disease underwent functional performance evaluations and muscle strength testing using handheld and electromechanical dynamometry, at baseline and 24 and 64 months after beginning enzyme replacement therapy.
Results: Quantitative improved functional performance and muscle strength were noted by this approach. Proximal muscle groups gained and retained more strength than did distal muscle groups.
Conclusions: This quantitative approach for tracking individual patient improvements shows promise for monitoring responsiveness to enzyme replacement therapy in late-onset Pompe disease.
Background: The cellular communications has been found to regulate numerous microbial events and plays a crucial role in elicitating microbial diversity within the population and overall changes in the surrounding environment. The presence and role of species-specific and inter-species microorganism cell to cell communication and the intrinsic networking of communication within an organization has been recently accepted. It has now been widely accepted that the cellular communications within the microbial system plays an important role in the healthy well-being and as well as in networking.
Objective: The mammalian digestive tract contains rich and diverse microbiota on its length. However, extensive studies have been reported on the role and variety of microorganism communities within the gut, scanty work has been reported on QS within the commensal and pathogenic microflora of the tract, with reference to the mode of pathogenesis and infection within the GI tract. The sole patent as of today related to the role of QS in GI tract is said to be on the detection of Lactobacillus strain within the GI Tract.
Conclusion: The potential role for QS in GI infections and interaction with commensal microflora is also a very important strategy for the hindrance and treatment.
Natali Calças, Ligia Mendonça, Luciane Perez, Rosangela Ferreira, Carina Elisei, and Alinne Castro*
In worldwide there is an alarming prevalence of obesity, diabetes mellitus, cardiovascular disease among others metabolic syndromes. These diseases have consequences on quality and expectancy of life of population. Significant efforts are devoted to the development of effective strategies for to prevent the development of specific diseases and conceptually achieve the human metabolic homeostasis. Growing evidence suggests that diet is the environmental central drivers that can affect the genetic information altering the metabolic function and consequently defining the health or disease state of an individual. Indeed, it has only been in recent years that gut microbiota has been associated with development of metabolic dysfunction, where their structure and composition can affect the host metabolism through multiple pathways. Recently, is well established that altering the dietary habits may influence the gut microbiota leading an imbalanced, a phenomenon known as dysbiosis. When there is the disruption of gut homeostasis is expected that certain microorganisms are linked to the pathogenesis being associated with multiple diseases. For this reason, extensive efforts have been carried out to overcome these obstacles and particular interest is involving in the pivotal role of diet on human health. In this context, the currently review aims to provide an overview and shed some light on fundamental characteristics of how diet act at the gut microbiota level in modulating some disorders including irritable bowel syndrome, obesity, type 2 diabetes mellitus, celiac disease among others disorders and consequently to draw up alternative strategies to use the diet as a therapeutic target.
Noura M. Darwish*, Ahmed Sultan, and Ahmed Malki
Background: Aberrant activation of the Ras/Raf-1/extracellular-regulated kinase (ERK) pathway has been shown to be involved in the progression of hepatocellular carcinoma (HCC). However, the mechanism of dysregulation of ERK activation is poorly understood. This study aimed to investigate the inhibitory effect of novel organo-copper supramolecular coordination polymer (SCP) on invasive potential of hepatocellular carcinoma (HCC) cells and to explore the underlying mechanism. Chemoprevention could represent an important means to inhibit the process of hepatocarcinogenesis.
Methods: The inhibitory effect of SCP compound on hepatoma cells was evaluated by the examination of HCC chemically induced liver tissues and specific pathway inhibition was examined by immunohistochemistry.
Results: Recently, we identified the newly synthesized SCP as a potent inhibitor of the Ras/Raf-1/ERK pathway. In this study, we found that the expression levels of ERK1/2 in chemically induced HCC tissue was frequently decreased in response to SCP treatment, comparing with those untreated with SCP compound. Moreover, ERK expression levels in HCC tissue were frequently correlated with the incidence of tumor invasion and metastasis. Increased expression of beta catenin in liver tissue, which was associated with increased ERK activation. P-ERK1/2 overexpression also reduced the expression of E-cadherin, which play important roles in tumor invasion and metastasis.
Conclusion: We concluded that SCP can effectively inhibit the invasive potential of ERK signaling pathway and ERK could be not only a novel prognostic factor but also a new therapeutic target for human HCC.
Abstract: Research in tissue engineering related to the improved processes using nanofiber scaffolds has seen considerable progress in the last decade in the regeneration and construction of a number of artificial tissue types. These designs are generally viewed from the perspective of possible sources for clinical implant and transplant materials. Nowadays, advancement in engineering of tissues often referred to as three-dimensional (3D) cell culture provides enhanced activities owing to the 3D systems that readily imitate the in vivo setting for differentiated organs, than a typical 2D cell culture. Electro-spinning has been useful in producing nanofibrous scaffolds with large surface area and high pore volume that has the potential to mimic the morphology of a tissue extracellular matrix and hence promoting cell attachment, proliferation and differentiation. This review reports improved processes of tissue revitalization utilizing electrospunnanofibrous scaffolds. Different tissue engineering approaches including their advantages have been discussed. Also, various biomaterials from both synthetic and natural origin have been elaborated.