1 The condition Marfan syndrome results from the production of abnormal elastin,

Question

1 The condition Marfan syndrome results from the production of abnormal elastin, the main protein component of elastic fibers. Predict the potential consequences of defective elastic fibers. (Hint: Consider the organs and tissues in which elastic fibers are found. How would defective elastic fibers impair the functions of these organs and tissues? Copyright Morton Publishing Compan Exploring A&P; in the Laboratory Edition: 2 ag mead09@gmail.com Printed from www.cheg com 2 other diseases, called collagen vascular diseases, affect the protein collagen, the main component of collagen fibers. Predict the possible consequences of defective collagen fibers on specific organs and tissues. 3 How would the effects of a collagen vascular disease that affected only the collagen in reticular fibers differ from the Chegg effects seen with question 2? Why? Copyright Go Morton Publishing Company Exploring A&P; in the Laboratory Edition: 2 ag.mead09@gmail.com Printed from www.chegg.com 4 The formation of fibrocartilage is a common response to injury of hyaline cartilage, Do you think fibrocartilage would provide an articular surface (i.e., the cartilage in joints) as smooth as the original hyaline cartilage? Why or why not? Copyright Morton Publishing Comp Exploring A&P; in the Laboratory Edition 2

Explanation / Answer

1. Elastic fibers are found in the skin, lungs, arteries, veins, connective tissue proper, elastic cartilage, periodontal ligament, fetal tissue and other structures. The main components of elastic fibers, elastin and fibrillin-containing microfibrils play a structural and mechanical role in the arteries and their essential function is to provide elasticity and resilience to the tissues.

Scientific evidence suggests that defects in any of the components making up the elastic fibers have the potential to alter and impair the appearance of these fibers. These alterations can lead to a variety of medical conditions, causing a variety of symptoms and complications. While certain cases of heart defects and hypertension, or high blood pressure, can be traced to abnormalities in these fibers, several lesser known conditions are a result of these defects as well.

There is evidence to believe that certain defects of any components of the elastic matrix may impair and alter the structural appearance of elastic and collagen fibers.

Such as in Williams syndrome , elastic matrix are defected with alterations in the elastin gene.

In Alpha-1 antitrypsin deficiency, a genetic disorder, elastin is excessively degraded by elastase, a degrading protein released by neutrophils during the inflammatory response. This leads most often to emphysema and liver disease in affected individuals.

Hypertension and some congenital heart defects are associated with alterations in the great arteries, arteries, and arterioles with alterations in the elastic matrix.

2. Collagen is one of the long, fibrous structural proteins. When they exists in bundles are known as collagen fibers which are a major component of the extracellular matrix that supports most tissues and gives cells structure from the outside. Sometimes collagen is also found inside certain cells. Collagen has great tensile strength, and is the main component of fascia, cartilage, ligaments, tendons, bone and skin. Along with elastin and soft keratin, it is responsible for skin strength and elasticity, and its degradation leads to wrinkles that accompany aging. It strengthens blood vessels and plays a role in tissue development. It is present in the cornea and lens of the eye in crystalline form.

One thousand mutations have been identified in 12 out of more than 20 types of collagen. These mutations can lead to impair the function of tissue and organ –

3. Collagen vascular disease is an autoimmune disease where immune system mistakenly attacks body’s healthy tissue. The attacks usually cause inflammation in collagen and nearby joints. People with collagen vascular disorders typically experience: fatigue, muscle weakness, fever, body aches, joint pain, and skin rash.

4. Hyaline cartilage is the smooth, glassy articular cartilage that normally covers the surface of joints. If hyaline cartilage is torn fibrocartilage will help to fill in the torn part of the cartilage. Fibrocartilage is a tough, dense, and fibrous material not smooth as the hyaline cartilage.

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