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Osteocytes, the most common cell type within mature cortical bone, actively participate in the growth and maintenance of TCVs through the transfer of mitochondria to endothelial cells. Scanning electron microscopy images have revealed that osteocytes possess numerous dendritic processes with expanded, endfoot-like structures.
MT-CO2 plays an essential role in the transfer of electrons from cytochrome c to the bimetallic center of the catalytic subunit 1 by utilizing its binuclear copper A center. It contains two adjacent transmembrane regions in its N-terminus and the major part of the protein is exposed to the periplasmic or to the mitochondrial intermembrane space ...
The mitochondrial diseases are genetic disorders carried in mitochondrial DNA, or nuclear DNA coding for mitochondrial components. Slight problems with any one of the numerous enzymes used by the mitochondria can be devastating to the cell, and in turn, to the organism.
MT-ND2 is located in mitochondrial DNA from base pair 4,470 to 5,511. [5] The MT-ND2 gene produces a 39 kDa protein composed of 347 amino acids. [10] [11] MT-ND2 is one of seven mitochondrial genes encoding subunits of the enzyme NADH dehydrogenase (ubiquinone), together with MT-ND1, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, and MT-ND6.
Mutations of DNA coding for mitochondrial membrane transport proteins are linked to a wide range of diseases and disorders, such as cardiomyopathy, encephalopathy, muscular dystrophy, epilepsy, neuropathy, and fingernail dysplasia. [44] Most mutations of mitochondrial membrane transporters are autosomal recessive.
MT-ND5 is located in mitochondrial DNA from base pair 12,337 to 14,148. [5] The MT-ND5 gene produces a 67 kDa protein composed of 603 amino acids. [ 9 ] [ 10 ] MT-ND5 is one of seven mitochondrial genes encoding subunits of the enzyme NADH dehydrogenase (ubiquinone) , together with MT-ND1 , MT-ND2 , MT-ND3 , MT-ND4 , MT-ND4L , and MT-ND6 .
Mitochondrial myopathies (MM) refer to a group of clinically and biochemically heterogeneous disorders that share common features of major mitochondrial structural abnormalities in skeletal muscle. The major morphological hallmark of MM is ragged, red fibers containing peripheral and intermyofibrillar accumulations of abnormal mitochondria.
MT-TP mutations may result in complex I deficiency of the mitochondrial respiratory chain, which may cause a wide variety of signs and symptoms affecting many organs and systems of the body, particularly the nervous system, the heart, and the muscles used for movement (skeletal muscles). These signs and symptoms can appear at any time from ...