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LIPID METABOLISM
LIPID METABOLISM

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Full Text PDF - Mary Ann Liebert, Inc. publishers
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... (VLCFA, C ‡ 22:0) and VLCFA-CoA esters into the peroxisome for degradation (66). Defective function of the ABCD1 transporter leads to VLCFA accumulation in most organs and plasma; and elevated levels of VLCFA are used as a biomarker for the biochemical diagnosis of the disease. Classical inactivatio ...
electron transport chain
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... combination of the reactions catalyzed by α-ketoglutarate dehydrogenase and succinyl-CoA synthetase). Initially, the energy released during the oxidation step is captured to form a thioester (covalently linked to glyceraldehyde-3-P dehydrogenase in glycolysis; succinyl-CoA in the TCA cycle). This th ...
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Mitochondrion



The mitochondrion (plural mitochondria) is a double membrane-bound organelle found in most eukaryotic cells. The word mitochondrion comes from the Greek μίτος, mitos, i.e. ""thread"", and χονδρίον, chondrion, i.e. ""granule"" or ""grain-like"".Mitochondria range from 0.5 to 1.0 μm in diameter. A considerable variation can be seen in the structure and size of this organelle. Unless specifically stained, they are not visible. These structures are described as ""the powerhouse of the cell"" because they generate most of the cell's supply of adenosine triphosphate (ATP), used as a source of chemical energy. In addition to supplying cellular energy, mitochondria are involved in other tasks, such as signaling, cellular differentiation, and cell death, as well as maintaining control of the cell cycle and cell growth. Mitochondria have been implicated in several human diseases, including mitochondrial disorders, cardiac dysfunction, and heart failure. A recent University of California study including ten children diagnosed with severe autism suggests that autism may be correlated with mitochondrial defects as well.Several characteristics make mitochondria unique. The number of mitochondria in a cell can vary widely by organism, tissue, and cell type. For instance, red blood cells have no mitochondria, whereas liver cells can have more than 2000. The organelle is composed of compartments that carry out specialized functions. These compartments or regions include the outer membrane, the intermembrane space, the inner membrane, and the cristae and matrix. Mitochondrial proteins vary depending on the tissue and the species. In humans, 615 distinct types of protein have been identified from cardiac mitochondria, whereas in rats, 940 proteins have been reported. The mitochondrial proteome is thought to be dynamically regulated. Although most of a cell's DNA is contained in the cell nucleus, the mitochondrion has its own independent genome. Further, its DNA shows substantial similarity to bacterial genomes.
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