Endoplazmik Retikulumda Katlanmamış Protein Cevabı ile İlişkili Hastalıklar

Abstract

The endoplasmic reticulum (ER) is the first compartment of the secretory pathway and is a bilayer membranous intracellular organelle found in almost all cells except highly specialized eukaryotic cells such as erythrocytes and sperm cells. The ER is involved in many different cellular functions. ER contributes to the production and folding of approximately one-third of cellular proteins, and plays an important role for the maintenance of cellular homeostasis. In addition to, it acts as a protein synthesis factory, contributes to the storage and regulation of calcium, to the synthesis and storage of lipids, and glucose metabolism. Besides these features, specific ER stress signaling pathways, known as the 'unfolded protein response', are required for maintaining ER homeostasis. ER homeostasis can be given cause for many pathological events, including protein misfolding or accumulation of mutant protein, which can lead to ER stress. In this review, unfolded protein response of the endoplasmic reticulum and diseases associated with unfolded protein response are discussed.