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  • ISSN: 2333-7109
    Current Issue
    Volume 4, Issue 1
    Case Report
    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.
    SzentgyorgyiReka Theresia, Laccone Franco, Rosigkeit Jens, Kopsa Wolfgang, and Kircher Susanne Gerit*
    Introduction: We present the unexpected findings of a 59-years-old female patient who was admitted for an imaging investigation after the exclusion of rheumatoid arthritis and recurring carpal tunnel syndrome she was operated on both hands several years earlier.
    Case presentation: Magnetic resonance imaging of the cervical spine revealed severe scoliosis in the cervicothoracic junction with biconcave-shaped vertebrae and dural thickening, especially in the craniocervical junction, leading to compression of the cervical spinal cord.
    The history of this patient detailed many therapeutic interventions, such as treatment of severe scoliosis during childhood, operations for a bilateral hallux valgus, replacement of the aortic and mitral valves, total hip endoprosthesis on one side and bilateral iridectomy due to glaucoma. She had severe hyperopia and hearing deficits on one side due to recurrent otitis media. Subsequent biochemical tests revealed an increased excretion of urinary glycosaminoglycans, which suggested an attenuated form of mucopolysaccharidosis. Moleculargenetic testing confirmed two combined heterozygous mutations in the ARSB-gene, which is responsible for mucopolysaccharidosis type VI -Maroteaux-Lamy syndrome. The diagnosis was made after the patient retired from working at a challenging full-time job for 41 years.
    Discussion: The key to the diagnosis was the finding of dural thickening in the craniocervical junction in the presence of severe cervical scoliosis and multiple ovoid-shaped vertebral bodies. The milder forms of mucopolysaccharidosis remain a challenge to diagnose them in adult patients. The confirmed diagnosis allowed the patient to be treated adequately with decompression by laminectomy and enzyme replacement therapy.
    Review Article
    Sriram Seshadri*
    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.
    Research Article
    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.
  • Current Issue Highlights
  • Over Expression of Novel Ubiquitin - Conjugating Enzyme Ube2q2l (E330021D16) Causes Cell Growth Delay/Arrest through Inducing DNA Damage
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