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Gene Review

ATP5AL1  -  ATP synthase, H+ transporting,...

Homo sapiens

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Disease relevance of ATP5AL1


Psychiatry related information on ATP5AL1


High impact information on ATP5AL1

  • Other structural and mutational probes of the F1 and F0 portions of the ATP synthase are reviewed, together with kinetic and other evaluations of catalytic site occupancy and behavior during hydrolysis or synthesis of ATP [9].
  • It lacks the pentose phosphate cycle and, more unexpectedly, ATP-synthase subunits, which are thought to be essential for life [10].
  • Different experimental approaches confirm this ectopic localization of components of the ATP synthase complex and the presence of ATP hydrolase activity at the hepatocyte cell surface [11].
  • We confirm this effect on endocytosis in perfused rat liver ex vivo by using a specific inhibitor of ATP synthase [11].
  • This has been confirmed in the direction of hydrolysis after isolation of the soluble F1 portion of the protein and visualization of the actual rotation of the central 'shaft' of the enzyme with respect to the rest of the molecule, making ATP synthase the world's smallest rotary engine [12].

Chemical compound and disease context of ATP5AL1


Biological context of ATP5AL1

  • We have now studied the second avian gene known to exist in a copy on the nonrecombining regions of both the Z and the W chromosome, viz., the ATP synthase alpha-subunit (ATP5A1) [18].
  • 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 [19].
  • The locations of the alpha and gamma 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 [19].
  • We report here that the amino acid sequence surrounding this residue is highly homologous to the beta-subunit of mitochondrial and bacterial ATP-synthase in the region of the polypeptide that is believed to contribute to nucleotide binding [20].
  • Homology between human bladder carcinoma oncogene product and mitochondrial ATP-synthase [20].

Anatomical context of ATP5AL1

  • The enzyme ATP synthase, or F-ATPase, is present in the membranes of bacteria, chloroplasts and mitochondria [21].
  • A 51.5-kD protein (p51.5) bearing structural and immunologic characteristics of the beta subunit of H+ transporting ATP synthase (E.C., beta-H+ATPase, published molecular mass of 51.6 kD) was detected on the plasma membrane of three different human tumor cell lines studied [22].
  • A novel ligand in lymphocyte-mediated cytotoxicity: expression of the beta subunit of H+ transporting ATP synthase on the surface of tumor cell lines [22].
  • Structural and immunogold-assisted assignment of two of these complexes, photosystem I (PS I) and ATP synthase, allowed direct determination of their surface density, which, for both, was found to be highest in grana margins [23].
  • The purified chloroplast ATP synthase (CF(0)-CF(1)) was reconstituted into azolectin liposomes from which bilayer membranes on the tip of a glass pipette ('dip stick technique')and planar bilayer membranes were form ed [24].

Associations of ATP5AL1 with chemical compounds


Physical interactions of ATP5AL1

  • The major storage component in CLN2 is the subunit c of mitochondrial ATP synthase complex and its accumulation is the direct result of lack of CLN2p in this disease [30].
  • Amino-terminal sequencing coupled with peptide mass fingerprinting and immunologic analyses identified the plasminogen binding protein as annexin II and the angiostatin binding protein as the alpha/beta-subunits of ATP synthase [31].
  • NRF-1 and NRF-2 can bind to some of the subunits of both CCO and ATP synthase to regulate gene expression [32].
  • Small-angle X-ray scattering studies of Mg.AT(D)P-induced hexamer to dimer dissociation in the reconstituted alpha 3 beta 3 complex of ATP synthase from thermophilic bacterium PS3 [33].
  • Purified AKAP121 KH domain binds the 3' untranslated regions (3'UTRs) of transcripts encoding the Fo-f subunit of mitochondrial ATP synthase and manganese superoxide dismutase (MnSOD) [34].

Enzymatic interactions of ATP5AL1


Co-localisations of ATP5AL1


Regulatory relationships of ATP5AL1


Other interactions of ATP5AL1


Analytical, diagnostic and therapeutic context of ATP5AL1


  1. A mutation in bilirubin uridine 5'-diphosphate-glucuronosyltransferase isoform 1 causing Crigler-Najjar syndrome type II. Bosma, P.J., Goldhoorn, B., Oude Elferink, R.P., Sinaasappel, M., Oostra, B.A., Jansen, P.L. Gastroenterology (1993) [Pubmed]
  2. Evidence that the Mg-dependent low-affinity binding site for ATP and Pi demonstrated on the isolated beta subunit of the F0.F1 ATP synthase is a catalytic site. Khananshvili, D., Gromet-Elhanan, Z. Proc. Natl. Acad. Sci. U.S.A. (1985) [Pubmed]
  3. Understanding and exploiting the mechanistic basis for selectivity of polyketide inhibitors of F(0)F(1)-ATPase. Salomon, A.R., Voehringer, D.W., Herzenberg, L.A., Khosla, C. Proc. Natl. Acad. Sci. U.S.A. (2000) [Pubmed]
  4. Immunologic identification of Na+,K(+)-ATPase isoforms in myocardium. Isoform change in deoxycorticosterone acetate-salt hypertension. Sweadner, K.J., Herrera, V.L., Amato, S., Moellmann, A., Gibbons, D.K., Repke, K.R. Circ. Res. (1994) [Pubmed]
  5. A novel deficiency of mitochondrial ATPase of nuclear origin. Houstek, J., Klement, P., Floryk, D., Antonická, H., Hermanská, J., Kalous, M., Hansíková, H., Hout'ková, H., Chowdhury, S.K., Rosipal, T., Kmoch, S., Stratilová, L., Zeman, J. Hum. Mol. Genet. (1999) [Pubmed]
  6. Apolipoprotein E associates with beta amyloid peptide of Alzheimer's disease to form novel monofibrils. Isoform apoE4 associates more efficiently than apoE3. Sanan, D.A., Weisgraber, K.H., Russell, S.J., Mahley, R.W., Huang, D., Saunders, A., Schmechel, D., Wisniewski, T., Frangione, B., Roses, A.D. J. Clin. Invest. (1994) [Pubmed]
  7. Abnormal levels of prohibitin and ATP synthase in the substantia nigra and frontal cortex in Parkinson's disease. Ferrer, I., Perez, E., Dalfó, E., Barrachina, M. Neurosci. Lett. (2007) [Pubmed]
  8. Artificial organelle: ATP synthesis from cellular mimetic polymersomes. Choi, H.J., Montemagno, C.D. Nano Lett. (2005) [Pubmed]
  9. The ATP synthase--a splendid molecular machine. Boyer, P.D. Annu. Rev. Biochem. (1997) [Pubmed]
  10. Reductive evolution suggested from the complete genome sequence of a plant-pathogenic phytoplasma. Oshima, K., Kakizawa, S., Nishigawa, H., Jung, H.Y., Wei, W., Suzuki, S., Arashida, R., Nakata, D., Miyata, S., Ugaki, M., Namba, S. Nat. Genet. (2004) [Pubmed]
  11. Ectopic beta-chain of ATP synthase is an apolipoprotein A-I receptor in hepatic HDL endocytosis. Martinez, L.O., Jacquet, S., Esteve, J.P., Rolland, C., Cabezón, E., Champagne, E., Pineau, T., Georgeaud, V., Walker, J.E., Tercé, F., Collet, X., Perret, B., Barbaras, R. Nature (2003) [Pubmed]
  12. Energy transduction in the F1 motor of ATP synthase. Wang, H., Oster, G. Nature (1998) [Pubmed]
  13. Inactivation of oxidized and S-nitrosylated mitochondrial proteins in alcoholic fatty liver of rats. Moon, K.H., Hood, B.L., Kim, B.J., Hardwick, J.P., Conrads, T.P., Veenstra, T.D., Song, B.J. Hepatology (2006) [Pubmed]
  14. Down-regulation of mitochondrial F1F0-ATP synthase in human colon cancer cells with induced 5-fluorouracil resistance. Shin, Y.K., Yoo, B.C., Chang, H.J., Jeon, E., Hong, S.H., Jung, M.S., Lim, S.J., Park, J.G. Cancer Res. (2005) [Pubmed]
  15. Membrane topology of subunit a of the F1F0 ATP synthase as determined by labeling of unique cysteine residues. Long, J.C., Wang, S., Vik, S.B. J. Biol. Chem. (1998) [Pubmed]
  16. Expression of chorionic gonadotropin-beta-like messenger ribonucleic acid in an alpha-subunit-secreting pituitary adenoma. Jameson, J.L., Lindell, C.M., Hsu, D.W., Habener, J.F., Ridgway, E.C. J. Clin. Endocrinol. Metab. (1986) [Pubmed]
  17. Human 3'-phosphoadenosine 5'-phosphosulfate synthetase (isoform 1, brain): kinetic properties of the adenosine triphosphate sulfurylase and adenosine 5'-phosphosulfate kinase domains. Lansdon, E.B., Fisher, A.J., Segel, I.H. Biochemistry (2004) [Pubmed]
  18. Male-biased mutation rates revealed from Z and W chromosome-linked ATP synthase alpha-subunit (ATP5A1) sequences in birds. Carmichael, A.N., Fridolfsson, A.K., Halverson, J., Ellegren, H. J. Mol. Evol. (2000) [Pubmed]
  19. Chromosomal localization of genes required for the terminal steps of oxidative metabolism: alpha and gamma subunits of ATP synthase and the phosphate carrier. Jabs, E.W., Thomas, P.J., Bernstein, M., Coss, C., Ferreira, G.C., Pedersen, P.L. Hum. Genet. (1994) [Pubmed]
  20. Homology between human bladder carcinoma oncogene product and mitochondrial ATP-synthase. Gay, N.J., Walker, J.E. Nature (1983) [Pubmed]
  21. Intersubunit rotation in active F-ATPase. Sabbert, D., Engelbrecht, S., Junge, W. Nature (1996) [Pubmed]
  22. A novel ligand in lymphocyte-mediated cytotoxicity: expression of the beta subunit of H+ transporting ATP synthase on the surface of tumor cell lines. Das, B., Mondragon, M.O., Sadeghian, M., Hatcher, V.B., Norin, A.J. J. Exp. Med. (1994) [Pubmed]
  23. From chloroplasts to photosystems: in situ scanning force microscopy on intact thylakoid membranes. Kaftan, D., Brumfeld, V., Nevo, R., Scherz, A., Reich, Z. EMBO J. (2002) [Pubmed]
  24. Single channel H+ currents through reconstituted chloroplast ATP synthase CF0-CF1. Wagner, R., Apley, E.C., Hanke, W. EMBO J. (1989) [Pubmed]
  25. PHLPP and a Second Isoform, PHLPP2, Differentially Attenuate the Amplitude of Akt Signaling by Regulating Distinct Akt Isoforms. Brognard, J., Sierecki, E., Gao, T., Newton, A.C. Mol. Cell (2007) [Pubmed]
  26. Mimicking photosynthetic solar energy transduction. Gust, D., Moore, T.A., Moore, A.L. Acc. Chem. Res. (2001) [Pubmed]
  27. H+/ATP ratio of proton transport-coupled ATP synthesis and hydrolysis catalysed by CF0F1-liposomes. Turina, P., Samoray, D., Gräber, P. EMBO J. (2003) [Pubmed]
  28. Alpha subunit variants of the human glycine receptor: primary structures, functional expression and chromosomal localization of the corresponding genes. Grenningloh, G., Schmieden, V., Schofield, P.R., Seeburg, P.H., Siddique, T., Mohandas, T.K., Becker, C.M., Betz, H. EMBO J. (1990) [Pubmed]
  29. The effect of nitric oxide on cell respiration: A key to understanding its role in cell survival or death. Beltrán, B., Mathur, A., Duchen, M.R., Erusalimsky, J.D., Moncada, S. Proc. Natl. Acad. Sci. U.S.A. (2000) [Pubmed]
  30. Biochemistry of neuronal ceroid lipofuscinoses. Junaid, M.A., Pullarkat, R.K. Adv. Genet. (2001) [Pubmed]
  31. Angiostatin binds ATP synthase on the surface of human endothelial cells. Moser, T.L., Stack, M.S., Asplin, I., Enghild, J.J., Højrup, P., Everitt, L., Hubchak, S., Schnaper, H.W., Pizzo, S.V. Proc. Natl. Acad. Sci. U.S.A. (1999) [Pubmed]
  32. Role of copper in mitochondrial biogenesis via interaction with ATP synthase and cytochrome c oxidase. Medeiros, D.M., Jennings, D. J. Bioenerg. Biomembr. (2002) [Pubmed]
  33. Small-angle X-ray scattering studies of Mg.AT(D)P-induced hexamer to dimer dissociation in the reconstituted alpha 3 beta 3 complex of ATP synthase from thermophilic bacterium PS3. Harada, M., Ito, Y., Sato, M., Aono, O., Ohta, S., Kagawa, Y. J. Biol. Chem. (1991) [Pubmed]
  34. PKA-dependent binding of mRNA to the mitochondrial AKAP121 protein. Ginsberg, M.D., Feliciello, A., Jones, J.K., Avvedimento, E.V., Gottesman, M.E. J. Mol. Biol. (2003) [Pubmed]
  35. The beta subunit of chloroplast ATP synthase (CF0CF1-ATPase) is phosphorylated by casein kinase II. Kanekatsu, M., Saito, H., Motohashi, K., Hisabori, T. Biochem. Mol. Biol. Int. (1998) [Pubmed]
  36. ATP-2 interacts with the PLAT domain of LOV-1 and is involved in Caenorhabditis elegans polycystin signaling. Hu, J., Barr, M.M. Mol. Biol. Cell (2005) [Pubmed]
  37. The biology of mitochondrial uncoupling proteins. Rousset, S., Alves-Guerra, M.C., Mozo, J., Miroux, B., Cassard-Doulcier, A.M., Bouillaud, F., Ricquier, D. Diabetes (2004) [Pubmed]
  38. Haem oxygenase 1 gene induction by glucose deprivation is mediated by reactive oxygen species via the mitochondrial electron-transport chain. Chang, S.H., Garcia, J., Melendez, J.A., Kilberg, M.S., Agarwal, A. Biochem. J. (2003) [Pubmed]
  39. Paclitaxel induces apoptosis via protein kinase A- and p38 mitogen-activated protein-dependent inhibition of the Na+/H+ exchanger (NHE) NHE isoform 1 in human breast cancer cells. Reshkin, S.J., Bellizzi, A., Cardone, R.A., Tommasino, M., Casavola, V., Paradiso, A. Clin. Cancer Res. (2003) [Pubmed]
  40. Upstream stimulatory factor 2 activates the mammalian F1F0 ATP synthase alpha-subunit gene through an initiator element. Breen, G.A., Jordan, E.M. Gene Expr. (1998) [Pubmed]
  41. Differential expression of mitochondria-encoded genes in a hibernating mammal. Hittel, D.S., Storey, K.B. J. Exp. Biol. (2002) [Pubmed]
  42. Comparative genomic hybridization analysis of Y79 and FISH mapping indicate the amplified human mitochondrial ATP synthase alpha-subunit gene (ATP5A) maps to chromosome 18q12-->q21. Godbout, R., Pandita, A., Beatty, B., Bie, W., Squire, J.A. Cytogenet. Cell Genet. (1997) [Pubmed]
  43. Human RNPS1 and its associated factors: a versatile alternative pre-mRNA splicing regulator in vivo. Sakashita, E., Tatsumi, S., Werner, D., Endo, H., Mayeda, A. Mol. Cell. Biol. (2004) [Pubmed]
  44. Regulation of the UGT1A1 bilirubin-conjugating pathway: Role of a new splicing event at the UGT1A locus. L??vesque, E., Girard, H., Journault, K., L??pine, J., Guillemette, C. Hepatology (2007) [Pubmed]
  45. Protein kinase A gating of a pseudopodial-located RhoA/ROCK/p38/NHE1 signal module regulates invasion in breast cancer cell lines. Cardone, R.A., Bagorda, A., Bellizzi, A., Busco, G., Guerra, L., Paradiso, A., Casavola, V., Zaccolo, M., Reshkin, S.J. Mol. Biol. Cell (2005) [Pubmed]
  46. ATP synthase: an electrochemical transducer with rotatory mechanics. Junge, W., Lill, H., Engelbrecht, S. Trends Biochem. Sci. (1997) [Pubmed]
  47. Abnormal Na+/H+ antiporter phenotype and turnover of immortalized lymphoblasts from type 1 diabetic patients with nephropathy. Ng, L.L., Davies, J.E., Siczkowski, M., Sweeney, F.P., Quinn, P.A., Krolewski, B., Krolewski, A.S. J. Clin. Invest. (1994) [Pubmed]
  48. RNA editing of wheat mitochondrial ATP synthase subunit 9: direct protein and cDNA sequencing. Bégu, D., Graves, P.V., Domec, C., Arselin, G., Litvak, S., Araya, A. Plant Cell (1990) [Pubmed]
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