Gene Symbol: HEM1
Description: 5-aminolevulinate synthase
Alias: CYD1, OLE3, 5-aminolevulinate synthase
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Gollub E, Liu K, Dayan J, Adlersberg M, Sprinson D. Yeast mutants deficient in heme biosynthesis and a heme mutant additionally blocked in cyclization of 2,3-oxidosqualene. J Biol Chem. 1977;252:2846-54 pubmed
    ..the pleiotropic properties of each of the mutants resulted from a single mutation in one of five unlinked loci (hem1 to hem5) affecting heme biosynthesis...
  2. Keng T, Alani E, Guarente L. The nine amino-terminal residues of delta-aminolevulinate synthase direct beta-galactosidase into the mitochondrial matrix. Mol Cell Biol. 1986;6:355-64 pubmed
    delta-Aminolevulinate synthase, the first enzyme in the heme biosynthetic pathway, is encoded by the nuclear gene HEM1. The enzyme is synthesized as a precursor in the cytoplasm and imported into the matrix of the mitochondria, where it ..
  3. SOTO I, Fontanesi F, Myers R, Hamel P, Barrientos A. A heme-sensing mechanism in the translational regulation of mitochondrial cytochrome c oxidase biogenesis. Cell Metab. 2012;16:801-13 pubmed publisher
    ..We conclude that heme sensing by Mss51 regulates COX biogenesis and aerobic energy production. ..
  4. Urban Grimal D, Labbe Bois R. Genetic and biochemical characterization of mutants of Saccharomyces cerevisiae blocked in six different steps of heme biosynthesis. Mol Gen Genet. 1981;183:85-92 pubmed
    ..These results are discussed in terms of possible modes of regulation of heme synthesis pathway in yeast. ..
  5. Köffel R, Schneiter R. Yeh1 constitutes the major steryl ester hydrolase under heme-deficient conditions in Saccharomyces cerevisiae. Eukaryot Cell. 2006;5:1018-25 pubmed
    ..These data thus provide the first link between fat degradation and the transcriptional control of lipase activity in yeast. ..
  6. Craven R, Mallory J, Hand R. Regulation of iron homeostasis mediated by the heme-binding protein Dap1 (damage resistance protein 1) via the P450 protein Erg11/Cyp51. J Biol Chem. 2007;282:36543-51 pubmed
  7. Protchenko O, Shakoury Elizeh M, Keane P, Storey J, Androphy R, Philpott C. Role of PUG1 in inducible porphyrin and heme transport in Saccharomyces cerevisiae. Eukaryot Cell. 2008;7:859-71 pubmed publisher
    ..The data from these experiments were consistent with a role for Pug1p in inducible protoporphyrin IX influx and heme efflux. ..
  8. Spanova M, Zweytick D, Lohner K, Klug L, Leitner E, Hermetter A, et al. Influence of squalene on lipid particle/droplet and membrane organization in the yeast Saccharomyces cerevisiae. Biochim Biophys Acta. 2012;1821:647-53 pubmed publisher
    ..that squalene, an intermediate of sterol biosynthesis, accumulates in yeast strains bearing a deletion of the HEM1 gene...
  9. Arrese M, Carvajal E, Robison S, Sambunaris A, Panek A, Mattoon J. Cloning of the ?-aminolevulinic acid synthase structural gene in yeast. Curr Genet. 1983;7:175-83 pubmed publisher
    b>HEM1, the structural gene for ?-aminolevulinic acid synthase, has been isolated on recombinant plasmids. A yeast genomic pool constructed in the E. coli - yeast shuttle vector YEp13 was used to clone the HEM1 gene by complementation...

More Information


  1. Kardon J, Yien Y, Huston N, Branco D, Hildick Smith G, Rhee K, et al. Mitochondrial ClpX Activates a Key Enzyme for Heme Biosynthesis and Erythropoiesis. Cell. 2015;161:858-67 pubmed publisher
    ..mtClpX, therefore, is a widely conserved stimulator of an essential biosynthetic pathway and uses a previously unrecognized mechanism for AAA+ unfoldases. ..
  2. Volland C, Felix F. Isolation and properties of 5-aminolevulinate synthase from the yeast Saccharomyces cerevisiae. Eur J Biochem. 1984;142:551-7 pubmed
    ..3 and a pH optimum of 7.4. Pyridoxal 5'-phosphate has been shown to be an essential cofactor. The enzyme activity was sensitive to thiol blocking reagents. Hemin, but not heme, inhibited the activity of the purified enzyme. ..
  3. Lin M, Unden H, Jacquier N, Schneiter R, Just U, Höfken T. The Cdc42 effectors Ste20, Cla4, and Skm1 down-regulate the expression of genes involved in sterol uptake by a mitogen-activated protein kinase-independent pathway. Mol Biol Cell. 2009;20:4826-37 pubmed publisher
    ..Together, these observations suggest that PAKs translocate into the nucleus, where they modulate expression of sterol uptake genes via Sut1, thereby controlling sterol homeostasis. ..
  4. Marek M, Milles S, Schreiber G, Daleke D, Dittmar G, Herrmann A, et al. The yeast plasma membrane ATP binding cassette (ABC) transporter Aus1: purification, characterization, and the effect of lipids on its activity. J Biol Chem. 2011;286:21835-43 pubmed publisher
    ..These results suggest a direct interaction between Aus1 and PS that is critical for the activity of the transporter...
  5. Pineau L, Bonifait L, Berjeaud J, Alimardani Theuil P, Berges T, Ferreira T. A lipid-mediated quality control process in the Golgi apparatus in yeast. Mol Biol Cell. 2008;19:807-21 pubmed
  6. Pineau L, Colas J, Dupont S, Beney L, Fleurat Lessard P, Berjeaud J, et al. Lipid-induced ER stress: synergistic effects of sterols and saturated fatty acids. Traffic. 2009;10:673-90 pubmed publisher
    ..The present data are discussed in the context of human diseases that involve lipid deregulation. ..