메뉴 건너뛰기




Volumn 86, Issue 21-22, 2010, Pages 825-831

Disturbance of mitochondrial energy homeostasis caused by the metabolites accumulating in LCHAD and MTP deficiencies in rat brain

Author keywords

Brain bioenergetics; LCHAD deficiency; Long chain 3 hydroxy fatty acids; MTP deficiency

Indexed keywords

3 HYDROXYACYL COENZYME A DEHYDROGENASE; 3 HYDROXYDODECANOIC ACID; 3 HYDROXYMYRISTIC ACID; 3 HYDROXYPALMITIC ACID; FATTY ACID; GLUTAMIC ACID; LAURIC ACID; MALIC ACID; MITOCHONDRIAL PROTEIN; MITOCHONDRIAL TRIFUNCTIONAL PROTEIN; PYRUVIC ACID; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE PHOSPHATE; SUCCINIC ACID; UNCLASSIFIED DRUG;

EID: 77952673083     PISSN: 00243205     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.lfs.2010.04.003     Document Type: Article
Times cited : (26)

References (36)
  • 1
    • 0021110398 scopus 로고
    • Lipid-protein interactions of reconstituted membrane-associated adenosine triphosphatases
    • Abeywardena M.Y., Allen T.M., Charnock J.S. Lipid-protein interactions of reconstituted membrane-associated adenosine triphosphatases. Biochimica et Biophysica Acta 1983, 729:62-74.
    • (1983) Biochimica et Biophysica Acta , vol.729 , pp. 62-74
    • Abeywardena, M.Y.1    Allen, T.M.2    Charnock, J.S.3
  • 2
    • 0017201717 scopus 로고
    • Safranine as a probe of the mitochondrial membrane potential
    • Akerman K.E., Wikström M.K. Safranine as a probe of the mitochondrial membrane potential. FEBS Letters 1976, 68:191-197.
    • (1976) FEBS Letters , vol.68 , pp. 191-197
    • Akerman, K.E.1    Wikström, M.K.2
  • 3
    • 0017184389 scopus 로고
    • A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding
    • Bradford M.M. A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding. Analytical Biochemistry 1976, 7(72):248-254.
    • (1976) Analytical Biochemistry , vol.7 , Issue.72 , pp. 248-254
    • Bradford, M.M.1
  • 4
    • 0025065453 scopus 로고
    • Inhibitors of the ATP/ADP antiporter suppress stimulation of mitochondrial respiration and H+ permeability by palmitate and anionic detergents
    • Brustovetskyl N.N., Dedukhova V.I., Egoroval M.V., Mokhova E.N., Skulachev V.P. Inhibitors of the ATP/ADP antiporter suppress stimulation of mitochondrial respiration and H+ permeability by palmitate and anionic detergents. FEBS Letters 1990, 272:187-189.
    • (1990) FEBS Letters , vol.272 , pp. 187-189
    • Brustovetskyl, N.N.1    Dedukhova, V.I.2    Egoroval, M.V.3    Mokhova, E.N.4    Skulachev, V.P.5
  • 5
    • 0031914110 scopus 로고    scopus 로고
    • Simultaneous analysis of plasma free fatty acids and their 3-hydroxy analogs in fatty acid beta-oxidation disorders
    • Costa C.G., Dorland L., Holwerda U., de Almeida I.T., Poll-The B.T., Jakobs C., Duran M. Simultaneous analysis of plasma free fatty acids and their 3-hydroxy analogs in fatty acid beta-oxidation disorders. Clinical Chemistry 1998, 44:463-471.
    • (1998) Clinical Chemistry , vol.44 , pp. 463-471
    • Costa, C.G.1    Dorland, L.2    Holwerda, U.3    de Almeida, I.T.4    Poll-The, B.T.5    Jakobs, C.6    Duran, M.7
  • 6
    • 0034085995 scopus 로고    scopus 로고
    • Secondary respiratory chain defect in a boy with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: possible diagnostic pitfalls
    • Das A.M., Fingerhut R., Wanders R.J., Ullrich K. Secondary respiratory chain defect in a boy with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: possible diagnostic pitfalls. European Journal of Pediatrics 2000, 159(4):243-246.
    • (2000) European Journal of Pediatrics , vol.159 , Issue.4 , pp. 243-246
    • Das, A.M.1    Fingerhut, R.2    Wanders, R.J.3    Ullrich, K.4
  • 7
    • 0036140895 scopus 로고    scopus 로고
    • Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: clinical presentation and follow-up of 50 patients
    • den Boer M.E., Wanders R.J., Morris A.A., IJlst L., Heymans H.S., Wijburg F.A. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: clinical presentation and follow-up of 50 patients. Pediatrics 2002, 109(1):99-104.
    • (2002) Pediatrics , vol.109 , Issue.1 , pp. 99-104
    • den Boer, M.E.1    Wanders, R.J.2    Morris, A.A.3    IJlst, L.4    Heymans, H.S.5    Wijburg, F.A.6
  • 8
    • 77957003282 scopus 로고
    • Mitochondrial respiratory control and the polarographic measurement of ADP/O ratios
    • Academic Press, New York, R.W. Estabrook, M.E. Pullman (Eds.)
    • Estabrook R.W. Mitochondrial respiratory control and the polarographic measurement of ADP/O ratios. Methods in Enzymology 1967, 41-47. Academic Press, New York. R.W. Estabrook, M.E. Pullman (Eds.).
    • (1967) Methods in Enzymology , pp. 41-47
    • Estabrook, R.W.1
  • 12
    • 0033745706 scopus 로고    scopus 로고
    • Quantitative measurement of total and free 3-hydroxy fatty acids in serum or plasma samples: short-chain 3-hydroxy fatty acids are not esterified
    • Jones P.M., Burlina A.B., Bennett M.J. Quantitative measurement of total and free 3-hydroxy fatty acids in serum or plasma samples: short-chain 3-hydroxy fatty acids are not esterified. Journal of Inherited Metabolic Disease 2000, 23:745-750.
    • (2000) Journal of Inherited Metabolic Disease , vol.23 , pp. 745-750
    • Jones, P.M.1    Burlina, A.B.2    Bennett, M.J.3
  • 13
    • 0347931746 scopus 로고    scopus 로고
    • Effects of odd-numbered medium-chain fatty acids on the accumulation of long-chain 3-hydroxy-fatty acids in long-chain L-3-hydroxyacyl CoA dehydrogenase and mitochondrial trifunctional protein deficient skin fibroblasts
    • Jones P.M., Butt Y.M., Bennett M.J. Effects of odd-numbered medium-chain fatty acids on the accumulation of long-chain 3-hydroxy-fatty acids in long-chain L-3-hydroxyacyl CoA dehydrogenase and mitochondrial trifunctional protein deficient skin fibroblasts. Molecular Genetics and Metabolism 2003, 81(2):96-99.
    • (2003) Molecular Genetics and Metabolism , vol.81 , Issue.2 , pp. 96-99
    • Jones, P.M.1    Butt, Y.M.2    Bennett, M.J.3
  • 14
    • 0015989408 scopus 로고
    • Effects of phospholipid acyl chain fluidity, phase transitions, and cholesterol on (Na+, K+)-stimulated adenosine triphosphatase
    • Kimmelberg H., Pahadjopoulos D. Effects of phospholipid acyl chain fluidity, phase transitions, and cholesterol on (Na+, K+)-stimulated adenosine triphosphatase. Journal of Biological Chemistry 1974, 249:1071-1080.
    • (1974) Journal of Biological Chemistry , vol.249 , pp. 1071-1080
    • Kimmelberg, H.1    Pahadjopoulos, D.2
  • 15
    • 49149109956 scopus 로고    scopus 로고
    • Mitochondrial fatty-acid oxidation disorders
    • Kompare M., Rizzo W.B. Mitochondrial fatty-acid oxidation disorders. Seminars in Pediatric Neurology 2008, 15(3):140-149.
    • (2008) Seminars in Pediatric Neurology , vol.15 , Issue.3 , pp. 140-149
    • Kompare, M.1    Rizzo, W.B.2
  • 17
    • 0017087276 scopus 로고
    • Model for action of local anesthetics
    • Lee A.G. Model for action of local anesthetics. Nature 1976, 262:545-548.
    • (1976) Nature , vol.262 , pp. 545-548
    • Lee, A.G.1
  • 19
    • 17144413027 scopus 로고    scopus 로고
    • Involvement of mitochondrial inner membrane anion carriers in the uncoupling effect of fatty acids
    • Mokhova E.N., Khailova L.S. Involvement of mitochondrial inner membrane anion carriers in the uncoupling effect of fatty acids. Biochemistry (Mosc.) 2005, 70:159-163.
    • (2005) Biochemistry (Mosc.) , vol.70 , pp. 159-163
    • Mokhova, E.N.1    Khailova, L.S.2
  • 21
  • 23
    • 0000044868 scopus 로고    scopus 로고
    • Mitochondrial fatty acid oxidation disorders
    • New York: McGraw-Hill, New York, C.R. Scriver, A.L. Beaudet, W.S. Sly, D. Valle (Eds.)
    • Roe C.R., Ding J. Mitochondrial fatty acid oxidation disorders. The Metabolic and Molecular Bases of Inherited Disease 2001, 1909-1963. New York: McGraw-Hill, New York. C.R. Scriver, A.L. Beaudet, W.S. Sly, D. Valle (Eds.).
    • (2001) The Metabolic and Molecular Bases of Inherited Disease , pp. 1909-1963
    • Roe, C.R.1    Ding, J.2
  • 25
    • 0034691657 scopus 로고    scopus 로고
    • Comparative study on uncoupling effects of laurate and lauryl sulfate on rat liver and skeletal muscle mitochondria
    • Samartsev V.N., Simonyan R.A., Markova O.V., Mokhova E.N., Skulachev V.P. Comparative study on uncoupling effects of laurate and lauryl sulfate on rat liver and skeletal muscle mitochondria. Biochimica et Biophysica Acta 2000, 145:179-190.
    • (2000) Biochimica et Biophysica Acta , vol.145 , pp. 179-190
    • Samartsev, V.N.1    Simonyan, R.A.2    Markova, O.V.3    Mokhova, E.N.4    Skulachev, V.P.5
  • 27
    • 0343835826 scopus 로고    scopus 로고
    • Refsum disease diagnostic marker phytanic acid alters the physical state of membrane proteins of liver mitochondria
    • Schonfeld P., Struy H. Refsum disease diagnostic marker phytanic acid alters the physical state of membrane proteins of liver mitochondria. FEBS Letters 1999, 457:179-183.
    • (1999) FEBS Letters , vol.457 , pp. 179-183
    • Schonfeld, P.1    Struy, H.2
  • 28
    • 0003720078 scopus 로고    scopus 로고
    • McGraw-Hill, New York, C.R. Scriver, A.L. Beaudet, W.S. Sly, D. Valle (Eds.)
    • The Metabolic and Molecular Bases of Inherited Disease 2001, 3-45. McGraw-Hill, New York. 8 edition. C.R. Scriver, A.L. Beaudet, W.S. Sly, D. Valle (Eds.).
    • (2001) The Metabolic and Molecular Bases of Inherited Disease , pp. 3-45
  • 29
    • 0033387853 scopus 로고    scopus 로고
    • Anion carriers in fatty acid-mediated physiological uncoupling
    • Skulachev V.P. Anion carriers in fatty acid-mediated physiological uncoupling. Journal of Bioenergetics and Biomembranes 1999, 31:431-445.
    • (1999) Journal of Bioenergetics and Biomembranes , vol.31 , pp. 431-445
    • Skulachev, V.P.1
  • 31
    • 0033041968 scopus 로고    scopus 로고
    • Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency
    • Tyni T., Pihko H. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Acta Paediatrica 1999, 88:237-245.
    • (1999) Acta Paediatrica , vol.88 , pp. 237-245
    • Tyni, T.1    Pihko, H.2
  • 32
    • 0030273410 scopus 로고    scopus 로고
    • Pathology of skeletal muscle and impaired respiratory chain function in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency with the G1528C mutation
    • Tyni T., Majander A., Kalimo H., Rapola J., Pihko H. Pathology of skeletal muscle and impaired respiratory chain function in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency with the G1528C mutation. Neuromuscular Disorders 1996, 6:327-337.
    • (1996) Neuromuscular Disorders , vol.6 , pp. 327-337
    • Tyni, T.1    Majander, A.2    Kalimo, H.3    Rapola, J.4    Pihko, H.5
  • 33
    • 6344235193 scopus 로고    scopus 로고
    • Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency
    • Tyni T., Paetau A., Strauss A.W., Middleton B., Kivelä T. Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Pediatric Research 2004, 56:744-750.
    • (2004) Pediatric Research , vol.56 , pp. 744-750
    • Tyni, T.1    Paetau, A.2    Strauss, A.W.3    Middleton, B.4    Kivelä, T.5
  • 34
    • 33845443630 scopus 로고    scopus 로고
    • Inhibition of adenine nucleotide transport in rat liver mitochondria by long-chain acyl-coenzyme A beta-oxidation intermediates
    • Ventura F.V., Tavares de Almeida I., Wanders R.J. Inhibition of adenine nucleotide transport in rat liver mitochondria by long-chain acyl-coenzyme A beta-oxidation intermediates. Biochemical and Biophysical Research Communication 2006, 352(4):873-878.
    • (2006) Biochemical and Biophysical Research Communication , vol.352 , Issue.4 , pp. 873-878
    • Ventura, F.V.1    Tavares de Almeida, I.2    Wanders, R.J.3
  • 36
    • 27644437646 scopus 로고    scopus 로고
    • Differential inhibitory effect of long-chain acyl-CoA esters on succinate and glutamate transport into rat liver mitochondria and its possible implications for long-chain fatty acid oxidation defects
    • Ventura F.V., Ruiter J., Ijlst L., de Almeida I.T., Wanders R.J. Differential inhibitory effect of long-chain acyl-CoA esters on succinate and glutamate transport into rat liver mitochondria and its possible implications for long-chain fatty acid oxidation defects. Molecular Genetics and Metabolism 2005, 86(3):344-352.
    • (2005) Molecular Genetics and Metabolism , vol.86 , Issue.3 , pp. 344-352
    • Ventura, F.V.1    Ruiter, J.2    Ijlst, L.3    de Almeida, I.T.4    Wanders, R.J.5


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.