Figure 1
From: Mitochondrial Mutations in Cholestatic Liver Disease with Biliary Atresia
Schematic representation of the human mitochondrial DNA and the mitochondrial respiratory chain. (a) Comparison of the mtDNA copy number in plasma between BA and CC patients. The mtDNA copy numbers of CC patient group were slightly higher than those of BA patient group. Data represent the mean ± SE. ∗p < 0.05 versus BA. (b) The map of the human mitochondrial genome (NC_012920.1) with the protein-coding genes colored according to the complexes to which they contribute subunits, two ribosomal RNAs, 22 tRNAs and non-coding D-loop in white. Montage depicting the structural information currently available for the five complexes that together contribute to the mitocondrial oxidative phosphorylation machinery. (c) Electrophoresis of the amplified DNA fragments for mtDNA by PCR. M, λ/HindIII DNA marker; lane 1, PCR product 1 (3,580 bp); lane 2, PCR product 2 (5,548 bp); lane 3, PCR product 3 (4,447 bp); lane 4, PCR product 4 (5,591 bp). (d) The mammalian mitochondrial electron transport chain includes the proton-pumping enzymes complex I (NADH–ubiquinone oxidoreductase), complex III (cytochrome bc1) and complex IV (cytochrome c oxidase), that combined, generate proton motive force that in turn drives F1FO-ATP synthase. Each complex is embedded in the lipid bilayer with the mitocondrial-encoded subunits colored corresponding to the genome diagram. The structure of each respiratory complex is presented: complex I from Thermus thermophilus (protein databank (PDB) code 4HEA), complex II from porcine Sus scrofa (PDB 4YXD), complex III from bovine Bos taurus (PDB 1L0L), complex IV from bovine B. taurus (PDB 1OCC) and complex V from bovine B. taurus (PDB 5ARA). The iron-sulfur cofactors of complex I are depicted as orange and yellow spheres. IMS, intermembrane space.
