The condition Marfan syndrome is a genetic disorder that affects proper producti

Question

The condition Marfan syndrome is a genetic disorder that affects proper production of the extra-cellular matrix protein fibrillin-1. Fibrillin-1 is necessary for proper production of elastic tissue fibers in the extracellular matrix, though other matrix fibers of these tissues are not affected. When elastic fibers are not properly formed, certain organs are severely affected. In extreme cases, the most affected organs include the large blood vessels near the heart which become weak, inelastic and can tear open (this is very bad and can cause immediate death).

For Marfan syndrome, which organelles produce fibrillin-1?

Mitochondria

Rough ER

For Marfan syndrome, which cells produce the elastic fibers?

For Marfan syndrome, the tissue in blood vessels that is affected is most likely:

Explanation / Answer

Ans 1: Rough ER produces fibrillin-1. Fibrillin is a glycoproteinmade from elastic fibres in the connective tissues. The mutation in the FBN1 gene causes Marfan syndrome. This disease mailny affects the connective tissues which provide strength and elasticity to muscles, bones blood vessels and ligaments.

Ans 2 : Fibroblasts and the smooth muscle cells in the arteries produce elastic fibres. Elastic fibres are the protein bundles present in the extracellular fluid of connective tissues.

Ans 3: Marfan syndrome mainly affects the connective tissue in the body which provide strength and elasticity to muscles, bones blood vessels and ligaments and heart valves. Marfan syndrome also affect vision due to dislocated lens called as ectopia lentis in the eyes and the large blood vessel that dispense blood from the heart to parts of the body and causes aortic valve defects.

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