Illuminating Enzyme
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Illuminating Enzyme
Different pattern of cystic fibrosis in lungs
Cystic fibrosis is an inherited disease of the mucus glands that affects many body systems. The disorder's most common signs and symptoms include progressive damage to the respiratory system and chronic digestive system problems.
Mucus is a slippery substance that lubricates and protects the linings of the airways, digestive system, reproductive system, and other organs and tissues. In people with cystic fibrosis, the body produces mucus that is abnormally thick and sticky. This abnormal mucus can obstruct the airways, leading to severe problems with breathing and bacterial infections in the lungs. These infections cause chronic coughing, wheezing, and inflammation. Over time, mucus buildup and infections result in permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs.
Most people with cystic fibrosis also have digestive problems because thick, sticky mucus interferes with the function of the pancreas. The pancreas is an organ that produces insulin (a hormone that helps control blood sugar levels). It also makes enzymes that help digest food. In people with cystic fibrosis, mucus blocks the ducts of the pancreas, preventing these enzymes from reaching the intestines to aid digestion. Problems with digestion can lead to diarrhea, malnutrition, poor growth, and weight loss. Some babies with cystic fibrosis have meconium ileus, a blockage of the intestine that occurs shortly after birth.
Cystic fibrosis used to be considered a fatal disease of childhood. With improved treatments and better ways to manage the disease, many people with cystic fibrosis now live well into adulthood. Adults with cystic fibrosis experience medical problems affecting the respiratory, digestive, and reproductive systems. For example, most men with cystic fibrosis are unable to father children (infertile) because the tubes that carry sperm (the vas deferens) are blocked by mucus and do not develop properly. This condition is known as congenital bilateral absence of the vas deferens (CBAVD). Infertility is also possible, though less common, in women with cystic fibrosis.
How common is cystic fibrosis?
Cystic fibrosis is a common genetic disease within the Caucasian (white) population in the United States. The disease occurs in 1 in 2,500 to 3,500 Caucasian newborns. Cystic fibrosis is less common in other ethnic groups, affecting about 1 in 17,000 African Americans and 1 in 31,000 Asian Americans.
Cystic fibrosis (CF), as Bals et al. discuss in the other Perspective in this issue , is one of the most thoroughly understood genetic diseases. Thus it is frustrating that CF lung disease has been refractory to the onslaught of molecular/cellular research that has clarified so many other aspects of CF. But after many false starts, recent experiments are finally beginning to crack the shell of ignorance that surrounds CF lung disease. A series of innovative experiments, mainly from two laboratories, has revealed fascinating new aspects of normal and CF lung biology. Yet despite the care and resourcefulness of each group, their findings generate sharply contrasting views of CF lung disease that lead to opposite treatment strategies! Fortunately, this controversy is generating more light than heat and is illuminating the field to the ultimate benefit of CF clinical care.
CF lung disease
Healthy airways are sterile below the first bronchial division. Sterility is maintained, despite constant challenge from viruses and bacteria in the air that we breathe, by an elaborate hierarchy of defenses. Lung defenses are understood only in outline. The airway surfaces are covered with a thin film (∼30 μm) of airway surface liquid (ASL) consisting of a periciliary sol and a mucus gel that are propelled toward the mouth by coordinated ciliary beating. Thus, mucociliary clearance, aided by cough, cleans the airways mechanically. The ASL is not simply saltwater, but is instead a rich broth of proteases/antiproteases, oxidants/antioxidants, antibiotics, and antibodies that work together to inactivate or destroy pathogens without undue collateral damage to the lungs. These mucosal mechanisms are backstopped by cellular immune mechanisms involving dendritic cells (2), neutrophils, and macrophages that are recruited and coordinated by signaling molecules in the ASL.
Although the complex pulmonary defense system is understood in outline, details are lacking because of technical impediments that, together with the lack of appropriate animal models, help to explain why we are still struggling to understand CF lung disease. The pattern of CF lung disease is unlike that of any other lung disease. In CF, infections of the smaller airways are nearly universal, difficult and eventually impossible to eradicate, and almost invariably lethal. The types of organisms that most bedevil CF patients, such as Pseudomonas aeruginosa or Burkholderia cepacia, are innocuous in normal individuals — to such an extent that they are being sprayed into the environment for various biotechnical applications. Disease is most severe in the upper lobes and involves huge infiltrations of neutrophils.
Functions of CFTR
As explained in more detail by Bals et al., CFTR, the product of the gene that is defective in CF, is a member of the ATP Binding Cassette (ABC) family. ABC family members have diverse functions, including ATP-dependent transmembrane pumping of larger molecules, regulation of other membrane transporters, and ion conductance. CFTR displays at least two of these functions. CFTR is an anion channel that uses the energy of ATP hydrolysis to transit through conducting and non-conducting conformations. Ion channels are equally adept at allowing ions to flow either into or out of cells, enabling the CFTR anion channel to play crucial roles in both absorption and secretion. Thus the loss of CFTR-mediated anion conductance explains a wide range of CF symptoms, such as elevated sweat chloride (a defect in salt absorption by the sweat ducts) and meconium ileus (a defect in fluid secretion by intestinal crypt cells).
Cystic fibrosis (CF) is an autosomal recessive disorder affecting approximately 25,000 persons in the United States. While CF can affect many organ systems, respiratory failure eventually accounts for the vast majority of mortality seen in the disease. Accordingly, despite treatment, many patients will eventually become candidates for lung transplantation. Given the long wait times for transplantable organs, accurate predictors of prognosis are urgently needed to optimize CF patient management.
About the Author
Dr. Dau Lal Bohra
PhD Animal Microbiology
Super confusing Chemistry question, where should I even start ?
Firefly luciferase is the enzyme that allows fireflies to illuminate their abdomens. Because this light generation is an ATP-requiring reaction, firefly luciferase can be used to test for the presence of ATP. In this way, luciferase can test for the presence of life. The coupled reactions are ...
1) luciferin O2 <--> oxyluciferin light
2) ATP<--> AMP PPi
If the overall Delta G of the coupled reaction is -5.50 kJ/mol, what is the K of the first of the above reactions at 30 degrees C?
Recall that the Delta G for the hydrolysis of ATP to ADP is -32.5 kJ/mol, and for ATP to AMP it is -31.6 kJ/mol.
Thanks for all your help !!
-5.50 kJ/mol = x + (-32.5 kJ/mol) + (-31.6 kJ/mol)
x = 58.6 kJ/mol. This is DeltaG for the first reaction.
K = exp(-DeltaG/RT) = exp(-58.6/[(.00831)(303 K)] = 7.8 x 10^-11
LATENT FIGURE PROTOCOL_PAUL VANOUSE
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