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The mitochondrial unfolded protein response (UPR mt) is a cellular stress response related to the mitochondria. The UPR mt results from unfolded or misfolded proteins in mitochondria beyond the capacity of chaperone proteins to handle them. [1] The UPR mt can occur either in the mitochondrial matrix or in the mitochondrial inner membrane. [1]
A mitochondrion (pl. mitochondria) is an organelle found in the cells of most eukaryotes, such as animals, plants and fungi.Mitochondria have a double membrane structure and use aerobic respiration to generate adenosine triphosphate (ATP), which is used throughout the cell as a source of chemical energy. [2]
The outer membrane mitochondrial proteins carry out functions for mitochondrial biogenesis and integration between mitochondria and the cellular system. The outer membrane consists of two types of integral proteins, including proteins with transmembrane β-barrel and proteins with one or more α-helical membrane anchors.
Phosphate carrier protein, mitochondrial is a protein that in humans is encoded by the SLC25A3 gene. [5] [6] The encoded protein is a transmembrane protein located in the mitochondrial inner membrane and catalyzes the transport of phosphate ions across it for the purpose of oxidative phosphorylation.
Many MC proteins preferentially catalyze the exchange of one solute for another ().A variety of these substrate carrier proteins, which are involved in energy transfer, have been found in the inner membranes of mitochondria and other eukaryotic organelles such as the peroxisome and facilitate the transport of inorganic ions, nucleotides, amino acids, keto acids and cofactors across the membrane.
Mitochondrial DNA is the small circular chromosome found inside mitochondria. These organelles, found in all eukaryotic cells, are the powerhouse of the cell. [1] The mitochondria, and thus mitochondrial DNA, are passed exclusively from mother to offspring through the egg cell.
The dynamic nature of mitochondria is critical for function. Chen and Chan (2010) have discussed the molecular basis of mitochondrial fusion, its protective role in neurodegeneration, and its importance in cellular function. [8] The mammalian mitofusins Mfn1 and Mfn2, GTPases localized to the outer membrane, mediate outer-membrane fusion.
This ratio is variable and mitochondria from cells that have a greater demand for ATP, such as muscle cells, contain even more cristae. Cristae membranes are studded on the matrix side with small round protein complexes known as F 1 particles, the site of proton-gradient driven ATP synthesis. Cristae affect overall chemiosmotic function of ...
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