Answer the both question The condition Marfan syndrome is a genetic disorder tha

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Answer the both 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? Rough ER Free ribosomes Smooth ER Mitochondria 2 0.5 The condition Marfan syndrome is a genetic disorder that affects proper production of the extra-cellular matrix protein fibrilln- 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 cells produce the elastic fibers? Osteocytes Adipocytes Myocytes Fibroblasts

Explanation / Answer

1. Fibrillin 1 is a CALCIUM BINDING GLYCOPROTEIN which is a major structural component of myofibrils located in the extracellular matrix of the connective tissue. Fibrinillin 1 is produced by ROUGH ENDOPLASMIC RETICULUM

2. Elastin fibers are mainly produced by FIBROBLASTS

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