3-Hydroxy-3-methylglutaryl coenzyme A lyase: Targeting and processing in peroxisomes and mitochondria

Journal of Lipid Research

Volumn 40, Issue 1, 1999, Pages 70-75

  Ashmarina, Lyudmila I ^a   Pshezhetsky, Alexey V ^a   Branda, Steven S ^b   Isaya, Grazia ^b,c   Mitchell, Grant A ^a  

^a UNIVERSITY OF MONTREAL   (Canada)

^b YALE UNIVERSITY SCHOOL OF MEDICINE   (United States)

^c MAYO CLINIC   (United States)

Author keywords

Amino acid metabolism; Hydroxymethylglutaryl CoA; Inborn errors; Ketoacid lyases; Ketone bodies; Mitochondria; Peroxisomes

Indexed keywords

3 HYDROXY 3 METHYLGLUTARYL COENZYME A; KETONE BODY; LYASE; ORNITHINE CARBAMOYLTRANSFERASE;

AMINO ACID METABOLISM; ARTICLE; CELL STRAIN HEPG2; CONTROLLED STUDY; DENSITY GRADIENT CENTRIFUGATION; ENZYME ACTIVITY; ENZYME DEFICIENCY; ENZYME LOCALIZATION; FIBROBLAST; HUMAN; HUMAN CELL; KETOGENESIS; LIPID METABOLISM; LYMPHOBLAST; MITOCHONDRION; PEROXISOME; PRIORITY JOURNAL; ZELLWEGER SYNDROME;

EID: 0032948460     PISSN: 00222275     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (31)

1. Ashmarina, L. I., N. Rusnak, H. M. Miziorko, and G. A. Mitchell. 1994. 3-Hydroxy-3-methylglutaryl-CoA lyase is present in mouse and human liver peroxisomes. J. Biol. Chem. 269: 31929-31932.
2. Krisans, S. K. 1996. Cell compartmentalization of cholesterol biosynthesis. Ann. NY Acad. Sci. 804: 142-164.
3. Mitchell, G. A., S. Kassovska-Bratinova, Y. Boukaftane, M-F. Robert, S. P. Wang, L. Ashmarina, M. Lambert, P. Lapierre, and E. Potier. 1995. Medical aspects of ketone body metabolism. Clin. Invest. Med. 18: 193-216.
4. Mitchell, G. A., M-F. Robert, P. W. Hruz, G. Fontaine, C. E. Behnke, L. M. Mende-Mueller, S. Wang, K. Shaeppert, C. Lee, K. M. Gibson, and H. Miziorko. 1993. HMG-CoA lyase (HL): cloning of human and chicken liver HL cDNAs, and characterization of a mutation causing human HL deficiency. J. Biol. Chem. 268: 4376-4381.
5. Wang, S., J. H. Nadeau, A. Duncan, M-F. Robert, G. Fontaine, K. Schappert, K. R. Johnson, E. Zietkiewicz, P. Hruz, H. Miziorko, and G. A. Mitchell. 1993. 3-Hydroxy-3-methylglutaryl coenzyme A lyase (HL): cloning and characterization of a mouse liver HL cDNA and subchromosomal mapping of the human and mouse HL genes. Mamm. Genome. 4: 382-387.
6. Ashmarina, L. I., M-F. Robert, M-A. Elsliger, and G. A. Mitchell. 1996. Characterization of the HMG-CoA lyase precursor, a protein targeted to peroxisomes and mitochondria. Biochem. J. 315: 71-75.
7. Lazarow, P. B., and H. W. Moser. 1995. Disorders of peroxisome biogenesis. In The Metabolic and Molecular Bases of Inherited Disease. C. R. Scriver, A. L. Beaudet, W. S. Sly, and D. Valle, editiors. McGraw Hill, New York. 2287-2324.
8. Mori, M., T. Morita, F. Ikeda, Y. Amaya, M. Tatibana, and P. P. Cohen. 1987. Synthesis, intracellular transport, and processing of the precursors for mitochondrial ornithine transcarbamylase and carbamoyl-phosphate synthetase I in isolated hepatocytes. Proc. Natl. Acad. Sci. USA. 78: 6056-6060.
9. Laemmli, U. K. 1970. Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature. 227: 680-685.
10. Wanders, R., R. Schutgens, and P. Zoeters. 1988. 3-Hydroxy-3-methylglutaryl-CoA lyase in human skin fibroblasts: study of its properties and deficient activity in 3-hydroxy-3-methylglutaric aciduria patients using a simple spectrophotometric method. Clin. Chim. Acta. 171: 95-102.
11. Leighton, F., B. Poole, H. Beaufay, P. Baudhuin, J. W. Coffey, S. Fowler, and C. de Duve. 1968. The large scale separation of peroxisomes, mitochondria, and lysosomes from the livers of rats injected with Triton Wr-1339. J. Cell. Biol. 37: 482-513.
12. Shmidt, E. 1974. Methods of Enzymatic Analysis. 2 ed. Verlag-Chemie, Weinheim, Germany. 650-656.
13. Noltmann, E. A. 1966. Phosphoglucose isomerase. Methods Enzymol. 9: 557-505.
14. Bradford, M. M. 1976. A rapid and sensitive method for the quantitation of microgram quantities of proteins utilizing the principal of protein-dye binding. Anal. Biochem. 72: 248-254.
15. Isaya, G., and F. Kalousek. 1995. Mitochondrial intermediate peptidase. Methods Enzymol. 248: 556-567.
16. Wanders, R. J. A., and R. B. H. Schutgens. 1985. Peroxisomal matrix enzymes in Zellweger syndrome: activity and subcellular localization in liver. J. Inherited Metab. Dis. 8(suppl. 2): 151-152.
17. Raymond, Y., and G. C. Shore. 1981. Processing of the precursor for the mitochondrial enzyme, carbamyl phosphate synthetase. J. Biol. Chem. 256: 2087-2090.
18. Jaussi, R., P. Sonderegger, J. Flückiger, and P. Christen. 1982. Biosynthesis and topogenesis of aspartate aminotransferase isoenzymes in chicken embryo fibroblasts. J. Biol. Chem. 257: 13334-13340.
19. Fenton, W. A., A. M. Hack, D. Helfgott, and L. E. Rosenberg. 1984. Biogenesis of the mitochondrial enzyme methylmalonyl-CoA mutase. J. Biol. Chem. 259: 6616-6621.
20. Felipo, V., and S. Grisolia. 1986. Precursors of mitochondrial proteins are degraded in the cytosol at different rates. FEBS Lett. 209: 227-229.
21. Lazarow, P. B., M. Robbi, Y. Fujiki, and L. Wong. 1982. Biogenesis of peroxisomal proteins in vivo and in vitro. Ann. NY Acad. Sci. 386: 285-300.
22. Santos, M. J., T. Imanaka, H. Shio, G. M. Small, and P. B. Lazarow. 1988. Peroxisomal membrane ghosts in Zellweger syndrome - Aberrant Organelle Assembly. Science. 239: 1536-1538.
23. Santos, M. J., T. Imanaka, H. Shio, and P. B. Lazarow. 1988. Peroxisomal integral membrane proteins in control and Zellweger fibroblasts. J. Biol. Chem. 21: 10502-10509.
24. Suzuki, Y., N. Shimozawa, T. Orii, and T. Hashimoto. 1989. Major peroxisomal membrane polypeptides are synthesized in cultured skin fibroblasts from patients with Zellweger syndrome. Pediatr. Res. 26: 150-153.
25. Balfe, A., G. Hoefler, W. W. Chen, and P. A. Watkins. 1990. Aberrant subcellular localization of peroxisomal 3-ketoacyl-CoA thiolase in the Zellweger syndrome and rhizomelic chondrodysplasia punctata. Pediatr. Res. 27: 304-310.
26. Wendland, M., and S. Subramani. 1993. Presence of cytoplasmic factors functional in peroxisomal protein import implicates organelle-associated defects in several human peroxisomal disorders. J. Clin. Invest. 92: 2402-2468.
27. Walton, P. A., S. J. Gould, J. R. Feramisco, and S. Subramani. 1992. Transport of microinjected proteins into peroxisomes of mammalian cells: inability of Zellweger cell lines to import proteins with the SKL tripeptide peroxisomal targeting signal. Mol. Cell. Biol. 12: 531-541.
28. Hartl, F-U., and W. Neupert. 1990. Protein sorting to mitochondria: evolutionary conservations of folding and assembly. Science. 247: 930-937.
29. Dodt, G., and S. J. Gould. 1996. Multiple PEX genes are required for proper subcellular distribution and stability of Pex5p, the PTS 1 receptor: evidence that PTS1 protein import is mediated by a cycling receptor. J. Cell. Biol. 135: 1763-1774.
30. Danpure, C. J., and P. E. Purdue. 1994. Primary hyperoxaluria. In The Metabolic and Molecular Bases of Inherited Disease. C. R. Scriver, A. L. Beaudet, W. S. Sly, and D. Valle, editors. McGraw-Hill, New York. 2385-2426.
31. Sweetman, L., and J. C. Williams. 1994. Branched chain organic acidurias. In The Metabolic and Molecular Bases of Inherited Disease. C. R. Scriver, A. L. Beaudet, W. S. Sly, and D. Valle, editors. McGraw-Hill, New York. 1387-1422.