Amyloidosis is?
Correct Answer: Misfolding of protein
Description: Ans. is 'd' i.e., Misfolding of protein * Protein misfolding disorders (proteinopathies) are conditions in which some proteins become structurally abnormal and impair the function of cells and tissues.* Protein folding is abnormal and they fail to perform normally and may even gain toxic effects.* Amyloidosis is such a group of diseases in which abnormal protein, known as amyloid protein, is deposited in tissue.* Amyloid is a protein that has an alteration in its secondary structure which imparts it a particular insoluble form, called the beta-pleated sheet conformation.* There are about 30 different type of amyloidosis, each due to a specific protein misfolding. They are grouped into localized and systemic forms. The four most common types of systemic disease are light chain (AL), inflammation (AA), dialysis (Ab2M), and hereditary and old age (ATTR)* All amyloids have in common birefringence in cross-polarized light after staining with the dye Congo Red, as well as a fibrillar ultrastructure when viewed with an electron microscope* Amyloid material is composed of two components -a) Major component (95%) - Fibril amyloid protein.b) Minor components (5%) - P-component, proteoglycans and sulfated glycosaminoglycans.* Fibril amyloid protein is responsible for tissue dysfunction due to pressure effect, while Pcomponent contributes to amyloid deposition by stabilizing fibril amyloid protein and decreasing their clearance*
Category:
Pathology
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