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With respect to the MT-ATP6 reading frame (+3), the MT-ATP8 gene ends in the +1 reading frame with a TAG stop codon. The MT-ATP6 protein weighs 24.8 kDa and is composed of 226 amino acids . [ 8 ] [ 9 ] The protein is a subunit of the F 1 F o ATPase, also known as Complex V , which consists of 14 nuclear- and 2 mitochondrial-encoded subunits.
Location of the MT-CO1 gene in the human mitochondrial genome.MT-CO1 is one of the three cytochrome c oxidase subunit mitochondrial genes (orange boxes).. Cytochrome c oxidase I (COX1) also known as mitochondrially encoded cytochrome c oxidase I (MT-CO1) is a protein that is encoded by the MT-CO1 gene in eukaryotes. [6]
With respect to the reading frame (+1) of MT-ATP8, the MT-ATP6 gene starts on the +3 reading frame. The MT-ATP8 protein weighs 8 kDa and is composed of 68 amino acids . [ 7 ] [ 8 ] The protein is a subunit of the F 1 F o ATPase, also known as Complex V , which consists of 14 nuclear- and 2 mitochondrial-encoded subunits.
Schematic karyogram showing the human genome, with 23 chromosome pairs, and the human mitochondrial genome to scale at bottom left (annotated "MT").Its genome is relatively tiny compared to the rest, and its copy number per human cell varies from 0 (erythrocytes) [1] up to 1,500,000 ().
MT-ND1 is a gene of the mitochondrial genome coding for the NADH-ubiquinone oxidoreductase chain 1 (ND1) protein. [5] The ND1 protein is a subunit of NADH dehydrogenase , which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain . [ 6 ]
MT-ND4 is a gene of the mitochondrial genome coding for the NADH-ubiquinone oxidoreductase chain 4 (ND4) protein. [5] The ND4 protein is a subunit of NADH dehydrogenase (ubiquinone) , which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain . [ 6 ]
English: The human genes MT-ATP8 and MT-ATP6 located in the mitochondrial DNA and coding for the ATP synthase F0 subunits 8 and 6 overlap on 46 nucleotides. The DNA sequence of this region of the human mitochondrial genome is given in black (positions 8,525 to 8,580 in the sequence accession NC_012920).
Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, LCHAD deficiency. Mitochondrial trifunctional protein (MTP) is a protein attached to the inner mitochondrial membrane which catalyzes three out of the four steps in beta oxidation.