The terminal steps of oxidative phosphorylation include transport of phosphate and ADP into the mitochondrial matrix, synthesis of ATP in the matrix, and transport of the product ATP into the cytosol where it can be utilized to perform cellular work. Three nuclear genome encoded membrane proteins, namely, the phosphate carrier (PHC), the adenine nucleotide carrier (ANT), and the ATP synthase complex, consisting of at least 13 individual subunits, catalyze these reactions. The locations of the α and γ subunits of the mitochondrial ATP synthase complex and the mitochondrial phosphate carrier, PHC, on human chromosomes were determined using cloned rat liver cDNA as probes. Human homologues of the α subunit are on chromosomes 9 and 18, the γ subunit are on chromosomes 10 and 14, and the PHC was localized to chromosome 12.
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Breen GA (1988) Bovine liver cDNA clones encoding a precursor of the alpha-subunit of the mitochondrial ATP synthase complex. Biochem Biophys Res Commun 152:264–269
Catterall WA, Coty WA, Pedersen PL (1973) Adenosine triphosphatase from rat liver mitochondria. J Biol Chem 248:7427–7431
Chau CA, Evans MJ, Scarpulla RC (1992) Nuclear respiratory factor 1 activation sites in the genes encoding the γ-subunit of ATP synthase, eukaryotic initiation factor 2α, and tryosine aminotransferase. Specific interaction of purified NRF-1 with multiple target genes. J Biol Chem 267:6999–7006
Drwinga HL, Toji LH, Kim CH, Greene AE, Mulivor RA (1993) NIGMS human/rodent somatic cell hybrid mapping panels 1 and 2. Genomics 16:311–314
Dubois BL, Naylor SL (1993) Characterization of NIGHMS human/rodent somatic cell hybrid mapping panel 2 by PCR. Genomics 16:315–319
Dyer MR, Gay NJ, Powell SJ, Walker JE (1989) ATP synthase from bovine mitochondria: complementary DNA sequence of the mitochondrial import precursor of the γ-subunit and the genomic sequence of the mature protein. Biochemistry 28:3670–3680
Ferreira GC, Pratt RD, Pedersen PL (1989) Energy-linked anion transport: cloning, sequencing, and characterization of a full length cDNA encoding the rat liver mitochondrial proton/phosphate symporter. J Biol Chem 264:15628–15633
Garboczi DN, Fox AH, Gerring SL, Pedersen PL (1988) β subunit of rat liver mitochondrial ATP synthase: cDNA cloning, amino acid sequence, expression in Escherichia coli, and structural relationship to adenylate kinase. Biochemistry 27:553–560
Joziasse DH, Shaper JH, Jabs EW, Shaper NL (1991) Characterization of an α1→3-galactosyltransferase homologue on human chromosome 12 that is organized as a processed pseudogene. J Biol Chem 266:6991–6998
Kudoh J, Minoshima S, Fukuyama R, Maekawa M, Neckelmann NS, Wallace DC, Shimizu Y, Shimizu N (1989) Assignment of the ATP synthase beta subunit (ATPMB) gene to the p13-qter region of human chromosome 12 and two related sequences (ATPMBL1 and ATPMBL2) to chromosome 2 and 17. Cytogenet Cell Genet 51:1026
Lee JH, Garboczi DN, Thomas PJ, Pedersen PL (1990) Mitochondrial ATP synthase: cDNA cloning, amino acid sequence, overexpression, and properties of the rat liver α subunit. J Biol Chem 265:4664–4669
Li K, Warner C, Hodge J, Minoshima S, Kudoh J, Fukuyama R, Maekawa M, Shimizu Y, Shimizu N, Wallace D (1989) A human muscle adenine nucleotide translocator gene has four exons, is located on chromosome 4, and is differentially expressed. J Biol Chem 264:13998–14004
Pedersen PL, Carafoli E (1987) Ion motive ATPases I: ubiquity, properties, and significance to cell function. Trends Biochem Sci 12:146–150
Tomura H, Endo H, Kagawa Y, Ohta S (1990) Novel regulatory enhancer in the nuclear gene of the human mitochondrial ATP synthase β-subunit. J Biol Chem 265:6525–6527
Walker JE, Powell SJ, Viñas O, Runswick MJ (1989) ATP synthase from bovine mitochondria: complementary DNA seuence of the import precursor of a heart isoform of the α subunit. Biochemistry 28:4702–4708
Webster KA, Oliver NA, Wallace DC (1982) Assignment of an oligomycin-resistance locus to human chromosome 10. Somat Cell Genet 8:223–224
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Jabs, E.W., Thomas, P.J., Bernstein, M. et al. Chromosomal localization of genes required for the terminal steps of oxidative metabolism: α and γ subunits of ATP synthase and the phosphate carrier. Hum Genet 93, 600–602 (1994). https://doi.org/10.1007/BF00202832
- Oxidative Phosphorylation
- Oxidative Metabolism
- Nuclear Genome
- Adenine Nucleotide