Abstract Scleroderma (SSc) or systemic sclerosis is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases. Many features of SSc are well known including inflammation, small-vessel vasculopathy, and overexpression of Extracellular Matrix (EMC) protein and fibrosis of the skin, lungs, and other internal organs. This study looks at different molecules that play important roles in the biological pathways in Scleroderma. Peroxisome Proliferator Activated Receptor-gamma (PPAR-ᵞ) is involved in regulation of fibrosis and adipogenesis in SSc. PPAR-γ has been found to be low in several cell types in SSc patients and African American (AA) donors. It is believed that healthy AA donor’s monocytes have similar characteristics to SSc patients’ monocytes. High levels of Transforming Growth Factor Beta (TGFβ), Interleukin-4 (IL-4) and Interleukin-13 (IL-13) levels are responsible for the decreasing of PPAR-ᵞ expression in monocytes isolated from SSc patients and healthy AA donors. Caveolin-1inhibits pro-fibrotic monocytes functions by up-regulation of PPAR-ᵞ and inhibition of monocyte migration. Treatments of monocytes derived from SSc patients …show more content…
and AA donors with Caveolin-1 Scaffolding Domain (CSD) peptide, can increase the expression and function of PPAR-ᵞ while treatments with IL-4, IL-13 and TGF-β can decrease the expression and function of PPAR-γ. Fibrosis of the lungs is of great importance due to high levels and migration rates towards stromal cell-derived factor 1 (SDF-1) in bone marrow (BM). CSD inhibits these migration rates due to the increasing Cav-1levels. AA monocytes are deficient in their ability to undergo Adipogenesis due to low Cav-1 and PPAR-γ levels. SSc adipocytes were smaller than healthy adipocyte which supports what is seen with skin lipodystrophy in SSc. Introduction Scleroderma or systemic sclerosis (SSc) is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases.
The word “scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin. Hardening of the skin is one of the most visible manifestations of this disease. Many features of scleroderma are well known including inflammation, fibrosis of the skin, lungs, and other internal organs and small-vessel vasculopathy. Currently in the United States, about 500,000 people are diagnosed with Scleroderma and around the world 2.5 million are also diagnosed (Barnes and Mayes 2012). Scleroderma is a rare but incurable disease and patients with this disease usually have an eleven year life span after diagnostics. (Mayes et. al
2003). Scleroderma is characterized by the overexpression of Extracellular Matrix (ECM) Proteins. Scleroderma disease can further develop into a fibrotic condition that causes deformation of the lungs and other internal organs (Song et al., 2011). The most important molecule in the development of SSc are myofibroblast cells and it is uncontrolled myofibroblast production of ECM proteins during healing process what differentiates SSc from normal healing processes (Del Galdo et al. 2008). Myofibroblast are In SSc fibroblasts, overproduction of proteins such as collagen, fibronectin, and glycosoaminoglycans has been observed as well as other pro-fibrotic cytokines (Atamas et al 2003). The initial onset or origin of the disease is not clear to date (Ju et al., 2011). Recent studies have brought focus on a protein kinase molecule named Caveolin-1 (Cav-1) which has been observed to be down-regulated in several cell types of SSc patients (Tourkina et al., 2010, 11, 12). In another study, Wang et al. (2006) found a reduction of Cav-1 in lung tissue of Idiopathic Pulmonary Fibrosis patients (IPF), which is a type of fibrotic disease. Cav-1 is one of three known to date Caveolin molecules and is known to regulate several physiological processes such as membrane trafficking, endocytosis, and signal transduction in cell proliferation and apoptosis (Wang et al., 2006). In addition to this, Cav-1 has been shown to play an important role in the regulation of ECM protein production via regulation of TGF-β receptor degradation (Del Galdo et al., 2008). TGF-β is a potent pro-fibrotic cytokine that binds to TGF-β receptor and has also been attributed to cause changes in Fibroblasts phenotype resulting in selection of fibroblasts resistant to apoptosis (Atamas et al., 2003). In addition to affected TGF-β receptor degradation, low Cav-1 levels have been associated with the overexpression of CXCR-4 receptors in monocytes of patients with interstitial lung disease (SSc-ILD) (Tourkina et al., 2011). CXCR-4 receptor plays a major role in migration of cells towards the lungs. In patients with ILD, lung injury causes the upregulation of chemoattractant SDF-1 and monocytes with upregulated CXCR-4 follow SDF-1 concentration gradient and accumulate in lung tissue (Tourkina et al., 2011). This phenomenon is of great importance because monocytes serve as precursors of fibrocytes (Bucala, 1994). In the present study, a model of SSc disease was recreated in mice in order to observe any differences in Cav-1 and CXCR-4 expression between bleomycin treated mice bone marrow (BM) cells versus a control group and how these changes affect the migration rate of the BM cells towards SDF-1 chemoattractant. Additionally, analysis in the percentage of Fibrocyte population in lung tissue was done in control and experimental group mice in order to observe if the possible hypermigration of cells towards SDF-1 is associated with a greater percentage of Fibrocytes present in lung tissue of bleomycin treated mice. Given the major role of Cav-1 in the regulation of SSc and fibrotic regulation, this study also presents the effects in hypermigration towards SDF-1 when cells are treated with CSD (Caveolin-1 Scaffolding Domain) peptide. CSD is a 19 amino acid peptide that constitutes the site of Cav-1 where kinase binding and inhibition occurs (Tourkina et al., 2012) and is believed mimic the effect of Cav-1 causing an increase in Cav-1 function (Tourkina et al., 2008)
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There are approximately six types of EDS that have been distinguished but other types exist that are very uncommon. Classical, Hypermobile, Vascular, Kyphoscoliosis, Arthrochalasia, and Dermatosparaxis. Classical and Hypermobile make up over 90% of all reported cases of EDS. With the Classical type of EDS a person would have hyperextensible (stretchy) skin with widened atrophic scars and joint hypermobility. The skin is smooth and velvety with tissue fragility and easy brusability. Also evident are molluscoid pseudotumors (fleshy lesions associated with scars) frequently found over pressure points (e.g. elbows) and subcutaneous spheroids, which are commonly mobile and palpable on the forearms and shins. Complications of joint hypemobility include sprains, dislocation are common in the shoulder, patella and temporomandibular joints Muscle hypotonia and slower gross motor development also can occur It is inherited in an autosomal dominant manner (Clarke, D., Skrocki-Czerpak, K., Neumann-Potash, L.).
Barone, Eugene J., Judson C. Jones, and Joann E. Schaefer. "Hidradenitis Suppurativa." Skin Disorders. Philadelphia: Lippincott Williams & Wilkins, 2000. 21-25. Print.
There are various methods of diagnosing Raydaund syndrome. Cold water emersion is one method. In this method, patients’ hands are immersed in cold water to observe any clinical features. Another mode of diagnosis looks at medical conditions that are associated with Raynaud syndrome, such as CTD, scleroderma, and lupus. A third technique includes physical examination of the ulnar and radial vessels, nail folds in the capillaries, presence of digital inflammation, sclerodactyly (sleroderma, hardening of the skin, of the fingers and toes), or telangiectasia (chronic dilation of groups of capillaries
What is scoliosis? Scoliosis is a musculoskeletal disorder that causes the back to curve sideways like and “S” or a “C” and cause the body to lean to one side. Scoliosis can eventually if not looked into and not treated colid with your bodily organs like your heart, lungs, and kidneys. This can cause you to slump down into a hump and cause it hard to breath and do physical activity.
Makover, M. & Zieve, D. (2011, February 14). Systemic Lupus Erythematosus. National Center for Biotechnology Information. Retrieved July 14, 2012, from http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001471/
In the 1990s, however, Bastuji and Roujeau each proposed that erythema multiform major and Stevens-Johnson syndrome are 2 distinct disorders (Roujeau, 1997). They suggested that the diagnosis of erythema ...
Psoriasis is a disease that has been associated with humans since the beginning of time. It is a type of autoimmune disease that produces red, slivery, and scaly patches to appear on the skin. Autoimmune disease is a type of disease that occurs when the body’s immune system mistaken the body’s organs as foreign substance. Psoriasis is chronic and can affect people of all ages but it is not contagious. There are currently about 125 million people around the world suffering from psoriasis or about 3% of the world’s total population. This essay will discuss the importance, biochemistry, and the potential treatment options of psoriasis.
Oedema is when fluid collected in the spaces between the cells in the body. The fluid leaks out of the damaged cells. The fluid can not be drained with a needle and probably won't improve by taking water pills. The build up of the fluid causes the affected tissue to swell up. The swelling can happen in one part of the body. For example, when you can injured, or it can also be more general. Along with the swelling and puffiness of the skin, oedema can cause stiff joints, raised blood pressure and pulse rate, aching, tender limbs, skin discoloration, weight loss or weight gain, and areas of the skin that hold an imprint of your finger when pressed. Oedema can happen anywhere in the body, but it usually happens in the feet and ankles. This
Psoriasis is a chronic skin disorder, easily identified by its symptoms of white, scaly skin and red lesions, though not so easily cured or understood. In psoriasis, skin cells mature faster than the body can shed them, causing a buildup. Although there are many theories as to what the cause of such a disease might be – genetics, stress, or other triggers – no one is quite sure why the disease occurs, or what could be a possible way to fully cure it. In this essay we will explore the symptoms, types, and effects of this condition, and also some of the known treatments.
Scleroderma (SSc) is a chronic disease characterized by the overexpression of Extracellular Matrix (ECM) Proteins. Scleroderma disease can further develop into a fibrotic condition that causes deformation of the lungs and other internal organs (Song et al., 2011). The most important players in the development of SSc are myofibroblast cells and it is uncontrolled myofibroblast production of ECM proteins during healing process what differentiates SSc from normal healing processes (Del Galdo et al., 2008). In SSc fibroblasts, overproduction of proteins such as collagen, fibronectin, and glycosoaminoglycans has been observed as well as other pro-fibrotic cytokines (Atamas et al., 2003). The initial onset or origin of the disease is not clear
In 2002, There was a horrible illness which is called severe acute respiratory syndrome, the abbreviation is SARS. SARS is a horrible disease which spreads fast. However, during the time, people post some fake news online and says vinegar and Isatis root are good for the illness. Therefore, people start to become crazy and buy those things but those are not good for the disease. One more example of myself experience. Recently, I have come down with a case of eczema, something I had never experienced before. My aunt read about a cure for eczema online that said that I should wash with hot water because hot water stimulates blood flow and dissipates the eczema. To my dismay, the eczema spread all over my body and I suffered horribly. I had to
Relevant cell observational tissue cultural studies: Omega-6 fatty acids have anti-inflammatory properties by inhibiting the production of interleukins, chemokines which reduces atherosclerosis progression [80]. Omega-6 has an anti-inflammatory effect by regulating PGE2 production from macrophages which inhibits TNF-α synthesis [7] and lipoxins formation [9]. Likewise, two studies found that PPARα expression in pretreated cultured human endothelial cells and human aortic smooth-muscle cells were significantly expressing the
In today’s world with technology and information so readily available it can sometimes be difficult to tell what information is factual and what is questionable. Answering simple questions may become more troublesome or demanding when you have to brave numerous resources to conclude which resources have validity and which do not. To show this I conducted research from a valid and a non-valid source to try to answer a question in hopes of improving my mother’s way of life. My mother has had with psoriasis for as long as I can remember. I am a witness to her constant struggle due towith the pain and recurring inflation which is why I would like to answer the question; what is the best treatment for psoriasis?
Before we can hope to reason correctly, we need God’s grace to transform our minds” (Jones p.13). Growing up with a Catholic background, I never knew much about what theology was. I had heard stories about the study of theology, but never practiced the understanding of the things of God. I grew up in a traditional Catholic background and was baptized when I was very young. I had the support of my family to attend church and have a relationship with God. On my mom 's side, I am a 5th generation Catholic, my dad grew up Episcopalian. My dad later was baptized into the Catholic church and accepted Christ. Both my parents have influenced me into the belief of God and his teachings. I fell away from Christ a couple years ago and put