Cystic Fibroblasts

Cystic fibrosis is a continuous, genetic disease that causes persistent lung infections and limits the ability to breathe over a period of time (Cystic Fibrosis Foundation). When an individual has cystic fibrosis, they tend to have lots of various lung problems. The overall cause of their lung problems is related to basic problems with diffusion and osmosis in the large airways of the lungs. The non-working cystic fibrosis proteins do not allow salt or water to enter the air space, resulting in the mucus layer to be extremely concentrated and very sticky. When you focus on individuals without cystic fibrosis, you can see that they have a small layer of salt water in the big airways of their lungs. This layer of salt water lies underneath

Cystic Fibrosis (CF) is an autosomal recessive genetic disease that causes thickened mucus to form in the lungs, pancreas, and other organs. It affects a specific protein called Cystic Fibrosis Transmembrane Regulator (CFTR) that controls the normal movement of sodium, chloride, and water in and out of the cells within the body. Those diagnosed with CF have either too little or abnormal CFTR. When CFTR is absent or defective, the mucus usually secreted by the cells in the pulmonary airways, pancreatic ducts, and gastrointestinal tract become thickened, leading to obstructions, frequent infection, and loss of function in the affected organs (Cystic Fibrosis Symptoms, Causes & Risk Factors, 2018). According to the Cystic Fibrosis Foundation

CF is caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CTFR) gene, which encodes a chloride ion channel that regulates osmotic balance across the epithelium through the transport of sodium ions and water4. Mutations

Cystic Fibrosis (CF) is a genetic disorder in which mucus glands produce abnormally thick secretions. These secretions can lead to chronic infections of the lungs and eventually lead to obstruction of the pancreas, resulting in digestive enzyme deficiency, the liver is also sometimes affected. Secretions from the sweat and salivary glands of a CF patient frequently contain abnormally high amounts of sodium and chloride. Because the body produces a high amount of salt, a sweat test is generally used to diagnose the disorder.

Cystic Fibrosis is a genetic disease that causes the body’s lungs to generate a different type of mucus than a non-infected body would. The contaminated lungs will produce mucus that is thick and adhesive which clogs the lungs and leads to an unpleasant and abhorrent lung infection. CF also interferes with the pancreas, disallowing the digestive enzymes from breaking down and absorbing food in the intestine. This can result in low nutrition, feeble growth, excessive sweat production, difficulties in breathing, and sometimes lung disease. When producing extra sweat and mucus, the body loses salt. If too much salt is lost, it can cause abnormal heart rhythms, disturbance of minerals in the blood, and perhaps, shock.

Myofibrils are made up of long proteins that include myosin, titin, and actin while other proteins bind them together. These proteins are arranged into thin and thick filaments that are repetitive along the myofibril in sectors known as sarcomeres. The sliding of actin and myosin filaments along each other is when the muscle is contracting. Dark A-bands and light I-bands reappear along myofibrils. The alignment of myofibrils causes an appearance of the cell to look banded or striated. A myofibril is made up of lots of sarcomeres. As the sarcomeres contract individually the muscle cells and myofibrils shorten in length. The longitudinal section of skeletal muscle exhibits a unique pattern of alternating light and dark bands. The dark staining, A-bands possess a pale region in the middle called the H-zone. In the middle of the H-zone the M-line is found, that displays filamentous structures that can join the thick filaments. The light-staining bands also known as I-bands are divided by thin Z-line. These striated patterns appear because of the presence of myofibrils in the sarcoplasm (IUPUI, 2016).

Cystic fibrosis is a genetic disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator protein, which results in a defective chloride channels. Chloride is part of salt that helps transportation of water needed for thin, free flowing mucus. The main characterization of cystic fibrosis is thick, sticky mucus in the lungs, making breathing difficult and allowing pathogens to invade (Ratjen and Döring 2003). Airway mucus creates an iron limiting environment to prevent bacterial growth. However, Pseudomonas aeruginosa is a bacteria able to resist this airway mucus. This proposes a question as to why people with cystic fibrosis are prone to infection when exposed to the same pathogen as any healthy individuals. Research has suggested defective mucus clearance and biofilm formation could contribute to prolonged Pseudomonas aeruginosa infection in people with cystic fibrosis (Gi et al. 2015). Also, targeting cystic fibrosis transmembrane conductance regulator proteins to allow chloride transportation has been proven to decrease mucus volume and Pseudomonas aeruginosa infected tissue (Rowe et al. 2014). The above information suggests increased mucus volume causes prolonged infection in the lungs of cystic fibrosis patients. This paper will describe cystic fibrosis and will focus on the unanswered question as to why cystic fibrosis patients experience chronic infection in their lungs.

are pore forming proteins which allow the passage of ions that are charged into and out of the cell

There are many diseases that greatly affect our respiratory physiology; one of those diseases is cystic fibrosis. Cystic fibrosis, according to the National Institute of Health (2013), is an inherited disease of the secretory glands or exocrine glands. Not only does this disease affect our respiratory system, it takes a toll on our digestive system. The respiratory system includes the gas exchange from our external atmosphere and our internal environment. This gas exchange of oxygen and carbon dioxide through our atmosphere and lungs does play a roll with cystic fibrosis. Cystic fibrosis is inherited, it affects many parts of the body mainly the respiratory system, and there are many signs and symptoms to this overwhelming disease.

Cystic Fibrosis is a genetic disorder caused by a mutation in a gene called the cystic fibrosis transmembrane conductance regulator (CFTR). The inheritance pattern is autosomal recessive. This means, to have symptoms of CF, you must have two defective CFTR genes, by inheriting a mutant copy of the CFTR gene from both your mother and your father. If you inherit a single defective CFTR gene from either your mother or your father, you are a “carrier” of the disease, but do not show symptoms, since it is a recessive gene. However, you could pass the gene onto your child and they could end up having cystic fibrosis, even if you do not have it. The CFTR gene makes a protein that controls how much salt and water move in and out of the body’s cells.

Cystic Fibrosis (CF) is an autosomal recessive gene that causes a wide range of symptoms because there are over 1,000 changes or mutations that can occur within the cystic fibrosis transmembrane receptor (CFTR) protein. The CFTR protein is generally a chloride ion chain “regulated by cyclic adenosine monophosphate and therefore can act as a regulator of other electrolyte channels”(Grossman, S., & Grossman, L. 2005, p. 46). Typically this protein allows chloride ions to exit mucus-producing cells allowing water to flow in and thin the mucus. However, if the CFTR protein has been mutated, such as in cystic fibrosis, chloride ions cannot exit. This causes the mucus to thicken, become sticky, and obstruct the various channels it passes through. This build up of mucus also prevents bacteria from being cleaned from cells thoroughly increasing the patients risk for infections (Grossman, S., & Grossman, L. 2005). However, the severity of CF depends on whether the patients have complete or partial loss of the CFTR gene. If the person has the classic form of CF abnormalities of CFTR will commonly affect “…the respiratory, gastrointestinal, endocrine and metabolic, and genitourinary systems”(Schram, C. 2012). However, if people have atypical forms of CF their genetic disorder may only affect one of the organ systems and may not be found until the patient develops symptoms in their late childhood, early adolescence, or adulthood

Cystic Fibrosis is caused by a genetic defect in Chromosome 7. Chromosome 7 encodes the cystic fibrosis transmembrane conductance regulator, also known as CFTR. There are over 1,000 mutations of this gene causing cystic fibrosis, with each mutation manifesting as a different variation of disease onset and clinical presentation. The most common mutation is the loss of phenylalanine residue at deltaF508. The abnormal functioning CFTR causes impaired chloride transport and more viscous secretions. The defect causes dehydrated secretions in the respiratory tract and gastrointestinal tract. Being dehydrated, these secretions become more difficult to move throughout the body. Along with impaired

When a tissue experiences the disruption of normal anatomic structure and function, it forms a wound. The process of restoring injured tissue to its normal structure and function by proliferation of neighboring living cells is called tissue regeneration (Sorg & Reinke, 2012). However, when regenation cannot occurs, wound healing occurs by replacement with a connection tissue and formation of a scar (Porth, 2011). Wound healing process involves the restoration of the integrity of injured tissues. It consists of three continous or overlapping phases: inflammation, proliferation, and wound contraction and remodeling phases (Porth, 2011). Any interruption, prolongation, or changes in the course can lead to delayed wound healing. The inflammation phase starts at the time of injury with both cellular and vascular responses. this phase is critical because it prepares optimum environment for wound healing (Porth, 2011). During the vascular response, blood vessels constrict and blood clots form to reduce blood loss as well as to fill the tissue gap. After that these vessels dilate and increase capillary permeability to allow plasma and blood components to leak into the wound. The accumulation of fluid causes the wound to appear swollen, red, and warm to touch (Sorg & Reinke, 2012). Platelets and white blood cells release cytokines and growth factors to stimulate the collagen synthesis, activate the transformation of fibroblasts to myofibroblasts, start the angiogenesis, and support

Cystic Fibrosis – Is an organelle caused disease where the pancreas, lung and other mucus producing cells trap salt within the cell causing drying out of the mucus in the affected areas until it is so sticky that it clogs these organs. The organelle in question is the Endoplasmic Reticulum, this is a membranous sack which performs the synthesis of proteins such as CFTR. CFTR is an integral membrane protein which is found lining the cells that are tasked to produce mucus in specific organs.

Immunohistochemical analysis for smooth muscle actin (SMA) was done and the intensity of staining and staining location were considered.