메뉴 건너뛰기




Volumn 3, Issue 2, 2000, Pages 101-108

The fundamentals and practice of docosahexaenoic acid therapy in peroxisomal disorders

Author keywords

[No Author keywords available]

Indexed keywords

DOCOSAHEXAENOIC ACID;

EID: 0033928650     PISSN: 13631950     EISSN: None     Source Type: Journal    
DOI: 10.1097/00075197-200003000-00004     Document Type: Review
Times cited : (7)

References (77)
  • 2
    • 0013897667 scopus 로고
    • Peroxisomes (microbodies and related particles)
    • de Duve C, Baudhuin P. Peroxisomes (microbodies and related particles). Physiol Rev 1966; 46:323-357.
    • (1966) Physiol Rev , vol.46 , pp. 323-357
    • De Duve, C.1    Baudhuin, P.2
  • 4
    • 0000228425 scopus 로고    scopus 로고
    • Disorders of peroxisome biogenesis
    • Scriver CR, Beaudet AL, Sly WS, Valle D (editors). 7th CD-ROM edition. New York: McGraw-Hill; Records
    • Lazarow PB, Moser HW. Disorders of peroxisome biogenesis. In: The metabolic basis of inherited disease. Scriver CR, Beaudet AL, Sly WS, Valle D (editors). 7th CD-ROM edition. New York: McGraw-Hill; 1997. Records 38247-38973.
    • (1997) The Metabolic Basis of Inherited Disease , pp. 38247-38973
    • Lazarow, P.B.1    Moser, H.W.2
  • 7
    • 0018184473 scopus 로고
    • Adrenoleukodystrophy: Preliminary report of a connatal case, light- and electron microscopical, immunohistochemical and biochemical findings
    • Berl
    • Ulrich J, Hershkowitz N, Heits P, Sigrist T, Baerlocher P. Adrenoleukodystrophy: preliminary report of a connatal case, light-and electron microscopical, immunohistochemical and biochemical findings. Acta Neuropathol (Berl) 1978; 43:77-83.
    • (1978) Acta Neuropathol , vol.43 , pp. 77-83
    • Ulrich, J.1    Hershkowitz, N.2    Heits, P.3    Sigrist, T.4    Baerlocher, P.5
  • 8
    • 0022480922 scopus 로고
    • Neonatal adrenoleukodystrophy: New cases, biochemical studies, and differentiation from Zellweger and related peroxisomal poly dystrophy syndromes
    • Kelley RI, Datta NS, Dobyns WB, Hajra AK, Moser AB, Noetzel MJ, et al. Neonatal adrenoleukodystrophy: new cases, biochemical studies, and differentiation from Zellweger and related peroxisomal poly dystrophy syndromes. Am J Med Genet 1986; 23:869-901.
    • (1986) Am J Med Genet , vol.23 , pp. 869-901
    • Kelley, R.I.1    Datta, N.S.2    Dobyns, W.B.3    Hajra, A.K.4    Moser, A.B.5    Noetzel, M.J.6
  • 9
    • 0019966135 scopus 로고
    • Infantile phytanic acid storage disease: A possible variant of Refsum's disease: three cases, including ultrastructural studies of the liver
    • Scotto JM, Hadchouel M, Odievre M, Laudat MH, Saudubray JM, Dulac O, et al. Infantile phytanic acid storage disease: a possible variant of Refsum's disease: three cases, including ultrastructural studies of the liver. J Inherit Metab Dis 1982; 5:83-90.
    • (1982) J Inherit Metab Dis , vol.5 , pp. 83-90
    • Scotto, J.M.1    Hadchouel, M.2    Odievre, M.3    Laudat, M.H.4    Saudubray, J.M.5    Dulac, O.6
  • 11
    • 0031963520 scopus 로고    scopus 로고
    • Peroxisomal disorders: Genotype, phenotype, major neuropathologic lesions, and pathogenesis
    • Powers JM, Moser HW. Peroxisomal disorders: genotype, phenotype, major neuropathologic lesions, and pathogenesis. Brain Pathol 1998; 8:101-120.
    • (1998) Brain Pathol , vol.8 , pp. 101-120
    • Powers, J.M.1    Moser, H.W.2
  • 12
    • 0021265709 scopus 로고
    • The cerebro-hepato-renal (Zellweger) syndrome: Increased levels and impaired degradation of very long chain fatty acids, their use in prenatal diagnosis
    • Moser AE, Singh I, Brown FR III, Solish GI, Kelley RI, Benke PJ, Moser HW. The cerebro-hepato-renal (Zellweger) syndrome: increased levels and impaired degradation of very long chain fatty acids, their use in prenatal diagnosis. N Engl J Med 1984; 310:1141-1146.
    • (1984) N Engl J Med , vol.310 , pp. 1141-1146
    • Moser, A.E.1    Singh, I.2    Brown III, F.R.3    Solish, G.I.4    Kelley, R.I.5    Benke, P.J.6    Moser, H.W.7
  • 14
    • 0022518008 scopus 로고
    • Accumulation and defective β-oxidation of very long chain fatty acids in Zellweger's syndrome, adrenoleukodystrophy and Refsum's disease variants
    • Poulos A, Singh H, Paton B, Sharp P, Derwas N. Accumulation and defective β-oxidation of very long chain fatty acids in Zellweger's syndrome, adrenoleukodystrophy and Refsum's disease variants. Clin Genet 1986; 29:397-408.
    • (1986) Clin Genet , vol.29 , pp. 397-408
    • Poulos, A.1    Singh, H.2    Paton, B.3    Sharp, P.4    Derwas, N.5
  • 15
    • 0022446974 scopus 로고
    • Deficient activities and proteins of peroxisomal β-oxidation enzymes in infants with Zellweger syndrome
    • Suzuki Y, Orii T, Mori M, Tatibana M, Hashimoto T. Deficient activities and proteins of peroxisomal β-oxidation enzymes in infants with Zellweger syndrome. Clin Chim Acta 1986; 156:191-196.
    • (1986) Clin Chim Acta , vol.156 , pp. 191-196
    • Suzuki, Y.1    Orii, T.2    Mori, M.3    Tatibana, M.4    Hashimoto, T.5
  • 16
    • 0023180287 scopus 로고
    • Peroxisomal very long chain fatty acid β-oxidation in human skin fibroblasts: Activity in Zellweger syndrome and other peroxisomal disorders
    • Wanders RJA, van Roermund CWT, van Wijland MJA, Heikoop J, Schutgens RBH, Schram AW, et al. Peroxisomal very long chain fatty acid β-oxidation in human skin fibroblasts: activity in Zellweger syndrome and other peroxisomal disorders. Clin Chim Acta 1987; 166:255-263.
    • (1987) Clin Chim Acta , vol.166 , pp. 255-263
    • Wanders, R.J.A.1    Van Roermund, C.W.T.2    Van Wijland, M.J.A.3    Heikoop, J.4    Schutgens, R.B.H.5    Schram, A.W.6
  • 17
    • 0018569248 scopus 로고
    • Subcellular localization of acyl-coenzyme A: Dihydroxyacetone phosphate acyltransferase in rat liver peroxisomes (microbodies)
    • Hajra AK, Burke CL, Jones CL. Subcellular localization of acyl-coenzyme A: dihydroxyacetone phosphate acyltransferase in rat liver peroxisomes (microbodies). J Biol Chem 1979; 254:10896-10900.
    • (1979) J Biol Chem , vol.254 , pp. 10896-10900
    • Hajra, A.K.1    Burke, C.L.2    Jones, C.L.3
  • 18
    • 0020002346 scopus 로고
    • Glycerolipid biosynthesis in peroxisomes via de acyl dihydroxyacetone phosphate pathway
    • Hajra AK, Bishop JE. Glycerolipid biosynthesis in peroxisomes via de acyl dihydroxyacetone phosphate pathway. Ann NY Acad Sci 1982; 386:170-182.
    • (1982) Ann NY Acad Sci , vol.386 , pp. 170-182
    • Hajra, A.K.1    Bishop, J.E.2
  • 19
    • 0020574070 scopus 로고
    • Severe plasmalogen deficiency in tissues of infants without peroxisomes (Zellweger syndrome)
    • Heymans HSA, Schutgens RBH, Tan R, van den Bosch H, Borst P. Severe plasmalogen deficiency in tissues of infants without peroxisomes (Zellweger syndrome). Nature 1983; 306:69-70.
    • (1983) Nature , vol.306 , pp. 69-70
    • Heymans, H.S.A.1    Schutgens, R.B.H.2    Tan, R.3    Van Den Bosch, H.4    Borst, P.5
  • 20
    • 0021132819 scopus 로고
    • Deficiency of enzymes catalyzing the biosynthesis of glycerol-ether lipids in Zellweger syndrome: A new category of metabolic disease involving the absence of peroxisomes
    • Datta NS, Wilson GN, Hajra AK. Deficiency of enzymes catalyzing the biosynthesis of glycerol-ether lipids in Zellweger syndrome: a new category of metabolic disease involving the absence of peroxisomes. N Engl J Med 1984; 31:1080-1083.
    • (1984) N Engl J Med , vol.31 , pp. 1080-1083
    • Datta, N.S.1    Wilson, G.N.2    Hajra, A.K.3
  • 21
    • 0019296591 scopus 로고
    • Conversion of 3a,7a,12a-trihydroxy-5b-cholestanoic acid into cholic acid by rat liver peroxisomes
    • Pedersen JI, Gustafsson J. Conversion of 3a,7a,12a-trihydroxy-5b-cholestanoic acid into cholic acid by rat liver peroxisomes. FEBS Lett 1980; 121:345-348.
    • (1980) FEBS Lett , vol.121 , pp. 345-348
    • Pedersen, J.I.1    Gustafsson, J.2
  • 22
    • 0022357561 scopus 로고
    • Bile acid synthesis in rat liver peroxisomes: Metabolism of 26-hydroxycholesterol to 3b-hydroxy-5-cholenoic acid
    • Krisans SK, Thompson SL, Pena LA, Kok E, Javitt NB. Bile acid synthesis in rat liver peroxisomes: metabolism of 26-hydroxycholesterol to 3b-hydroxy-5-cholenoic acid. J Lipid Res 1985; 26:1324-1932.
    • (1985) J Lipid Res , vol.26 , pp. 1324-1932
    • Krisans, S.K.1    Thompson, S.L.2    Pena, L.A.3    Kok, E.4    Javitt, N.B.5
  • 24
    • 0021961770 scopus 로고
    • 3-Hydroxy-3-methylglutaryl coenzyme a reductase is present in peroxisomes in normal rat liver cells
    • Keller GA, Barton MC, Shapiro DJ, Singer SJ. 3-Hydroxy-3-methylglutaryl coenzyme A reductase is present in peroxisomes in normal rat liver cells. Proc Natl Acad Sci U S A 1985; 82:770-774.
    • (1985) Proc Natl Acad Sci U S A , vol.82 , pp. 770-774
    • Keller, G.A.1    Barton, M.C.2    Shapiro, D.J.3    Singer, S.J.4
  • 25
    • 0026932050 scopus 로고
    • The role of peroxisomes in cholesterol metabolism
    • Krisans SK. The role of peroxisomes in cholesterol metabolism. Am J Respir Cell Mol Biol 1992; 7:358-364.
    • (1992) Am J Respir Cell Mol Biol , vol.7 , pp. 358-364
    • Krisans, S.K.1
  • 26
    • 0021858767 scopus 로고
    • Cerebro-hepato-renal (Zellweger) syndrome, adrenoleukodystrophy and Refsum's disease; plasma changes and skin fibroblast phytanic acid oxidase
    • Poulos A, Sharp P, Fellenberg AJ, Danks DM. Cerebro-hepato-renal (Zellweger) syndrome, adrenoleukodystrophy and Refsum's disease; plasma changes and skin fibroblast phytanic acid oxidase. Hum Genet 1985; 70:172-177.
    • (1985) Hum Genet , vol.70 , pp. 172-177
    • Poulos, A.1    Sharp, P.2    Fellenberg, A.J.3    Danks, D.M.4
  • 27
    • 0025304695 scopus 로고
    • A comparative study of straight chain and branched chain fatty acid oxidation in skin fibroblasts from patients with peroxisomal disorders
    • Singh H, Usher S, Johnson D, Poulos A. A comparative study of straight chain and branched chain fatty acid oxidation in skin fibroblasts from patients with peroxisomal disorders. J Lipid Res 1990; 31:217-225
    • (1990) J Lipid Res , vol.31 , pp. 217-225
    • Singh, H.1    Usher, S.2    Johnson, D.3    Poulos, A.4
  • 28
    • 0016615916 scopus 로고
    • Cerebro-hepato-renal syndrome of Zellweger: A report of eight cases with comments upon the incidence, the liver lesion, and a fault in pipecolic acid metabolism
    • Danks DM, Tippett P, Adams C, Campbell P: Cerebro-hepato-renal syndrome of Zellweger: a report of eight cases with comments upon the incidence, the liver lesion, and a fault in pipecolic acid metabolism. J Pediatr 1975; 86:382-387.
    • (1975) J Pediatr , vol.86 , pp. 382-387
    • Danks, D.M.1    Tippett, P.2    Adams, C.3    Campbell, P.4
  • 29
    • 0022457221 scopus 로고
    • The significance of hyperpipecoliatemia in Zellweger syndrome
    • Dancis J, Hutzler J. The significance of hyperpipecoliatemia in Zellweger syndrome. Am J Hum Genet 1986; 38:707-711.
    • (1986) Am J Hum Genet , vol.38 , pp. 707-711
    • Dancis, J.1    Hutzler, J.2
  • 30
    • 0023548002 scopus 로고
    • Identification of a peroxisomal targeting signal at the carboxy terminus of firefly luciferase
    • Gould SG, Keller GA, Subramani S. Identification of a peroxisomal targeting signal at the carboxy terminus of firefly luciferase. J Cell Biol 1987; 105:2923-2931.
    • (1987) J Cell Biol , vol.105 , pp. 2923-2931
    • Gould, S.G.1    Keller, G.A.2    Subramani, S.3
  • 31
    • 0025941962 scopus 로고
    • A novel, cleavable peroxisomal targeting signal at the amino-terminus of the rat 3-ketoacyl-CoA thiolase
    • Swinkels BW, Gould SJ, Bodnar AG, Rachubinski RA, Subramani S. A novel, cleavable peroxisomal targeting signal at the amino-terminus of the rat 3-ketoacyl-CoA thiolase. EMBO J 1991; 10:3255-3262.
    • (1991) EMBO J , vol.10 , pp. 3255-3262
    • Swinkels, B.W.1    Gould, S.J.2    Bodnar, A.G.3    Rachubinski, R.A.4    Subramani, S.5
  • 33
    • 0031941276 scopus 로고    scopus 로고
    • Components involved in peroxisome import, biogenesis, proliferation, turnover, and movement
    • Subramani S. Components involved in peroxisome import, biogenesis, proliferation, turnover, and movement. Physiol Rev 1998; 78:171-188.
    • (1998) Physiol Rev , vol.78 , pp. 171-188
    • Subramani, S.1
  • 35
    • 0023932509 scopus 로고
    • Peroxisomal membrane ghosts in Zellweger syndrome - aberrant organelle assembly
    • Santos MJ, Imanaka T, Shio H, Small GM, Lazarow PB. Peroxisomal membrane ghosts in Zellweger syndrome - aberrant organelle assembly. Science 1988; 239:1536-1538.
    • (1988) Science , vol.239 , pp. 1536-1538
    • Santos, M.J.1    Imanaka, T.2    Shio, H.3    Small, G.M.4    Lazarow, P.B.5
  • 36
    • 0023879539 scopus 로고
    • Genetic heterogeneity in the cerebrohepatorenal (Zellweger) syndrome and other inherited disorders with a generalized impairment of peroxisomal functions - A study using complementation analysis
    • Brul S, Westerveld A, Strijland A, Wanders PJA, Schram AW, Heymans HSA, et al. Genetic heterogeneity in the cerebrohepatorenal (Zellweger) syndrome and other inherited disorders with a generalized impairment of peroxisomal functions - a study using complementation analysis. J Clin Invest 1988; 81:1710-1715.
    • (1988) J Clin Invest , vol.81 , pp. 1710-1715
    • Brul, S.1    Westerveld, A.2    Strijland, A.3    Wanders, P.J.A.4    Schram, A.W.5    Heymans, H.S.A.6
  • 37
    • 0024397520 scopus 로고
    • Genetic and phenotypic heterogeneity in disorders of peroxisome biogenesis - A complementation study involving cell lines from 19 patients
    • Roscher AA, Hoefler S, Hoefler G, Paschke E, Paltauf F, Moser A, Moser HW. Genetic and phenotypic heterogeneity in disorders of peroxisome biogenesis - a complementation study involving cell lines from 19 patients. Pediatr Res 1989; 26:67-72.
    • (1989) Pediatr Res , vol.26 , pp. 67-72
    • Roscher, A.A.1    Hoefler, S.2    Hoefler, G.3    Paschke, E.4    Paltauf, F.5    Moser, A.6    Moser, H.W.7
  • 38
    • 0026566323 scopus 로고
    • Complementation study of peroxisome-deficient disorders by immunofluorescence staining and characterization of fused cells
    • Yajima S, Suzuki T, Shimozawa N, Yamaguchi S, Orii T, Fujiki Y, et al. Complementation study of peroxisome-deficient disorders by immunofluorescence staining and characterization of fused cells. Hum Genet 1992; 88:491-499.
    • (1992) Hum Genet , vol.88 , pp. 491-499
    • Yajima, S.1    Suzuki, T.2    Shimozawa, N.3    Yamaguchi, S.4    Orii, T.5    Fujiki, Y.6
  • 39
    • 0342743782 scopus 로고
    • Phenotype of patients with peroxisomal disorders subdivided into sixteen complementation groups
    • Moser A, Rasmussen M, Naidu S, Watkins P, McGuinness M, Hajra A, et al. Phenotype of patients with peroxisomal disorders subdivided into sixteen complementation groups. J. Pediatr 1995; 125:755-767.
    • (1995) J. Pediatr , vol.125 , pp. 755-767
    • Moser, A.1    Rasmussen, M.2    Naidu, S.3    Watkins, P.4    McGuinness, M.5    Hajra, A.6
  • 40
  • 41
    • 7844239443 scopus 로고    scopus 로고
    • Temperature-sensitive mutation in PEX1 moderates the phenotypes of peroxisome deficiency disorders
    • Imamura A, Tamura S, Shimozawa N, Suzuki Y, Zhang Z, Tsukamoto T, et al. Temperature-sensitive mutation in PEX1 moderates the phenotypes of peroxisome deficiency disorders. Hum Mol Genet 1998; 7:2089-2094.
    • (1998) Hum Mol Genet , vol.7 , pp. 2089-2094
    • Imamura, A.1    Tamura, S.2    Shimozawa, N.3    Suzuki, Y.4    Zhang, Z.5    Tsukamoto, T.6
  • 42
    • 0032789807 scopus 로고    scopus 로고
    • A common PEX1 frameshift mutation in patients with disorders of peroxisome biogenesis correlates with the severe Zellweger syndrome phenotype
    • Maxwell MA, Nelson PV, Chin SJ, Paton BC, Carey WF, Crane DI. A common PEX1 frameshift mutation in patients with disorders of peroxisome biogenesis correlates with the severe Zellweger syndrome phenotype. Hum Genet 1999; 105:38-44. An important article that differentiates the classic Zellweger's syndrome phenotype from its milder variants at the molecular level.
    • (1999) Hum Genet , vol.105 , pp. 38-44
    • Maxwell, M.A.1    Nelson, P.V.2    Chin, S.J.3    Paton, B.C.4    Carey, W.F.5    Crane, D.I.6
  • 43
    • 0033061268 scopus 로고    scopus 로고
    • Identification of a common PEX1 mutation in Zellweger syndrome
    • Collins CS, Gould SJ. Identification of a common PEX1 mutation in Zellweger syndrome. Hum Mutat 1999; 14:45-53.
    • (1999) Hum Mutat , vol.14 , pp. 45-53
    • Collins, C.S.1    Gould, S.J.2
  • 44
    • 0026036765 scopus 로고
    • Developmental profiles of polyunsaturated fatty acids in the brain of normal infants and patients with peroxisomal diseases: Severe deficiency of docosahexaenoic acid in Zellweger's and pseudo-Zellweger's syndromes
    • Simopoulos AP (editor). Basilea: S. Karger
    • Martinez M. Developmental profiles of polyunsaturated fatty acids in the brain of normal infants and patients with peroxisomal diseases: severe deficiency of docosahexaenoic acid in Zellweger's and pseudo-Zellweger's syndromes. In: World review of nutrition and dietetics. Simopoulos AP (editor). Basilea: S. Karger; 1991. pp. 87-102.
    • (1991) World Review of Nutrition and Dietetics , pp. 87-102
    • Martinez, M.1
  • 45
    • 0026680612 scopus 로고
    • Phospholipids in X-linked adrenoleukodystrophy white matten fatty acid abnormalities before the onset of demyelination
    • Theda C, Moser AB, Powers JM, Moser HW. Phospholipids in X-linked adrenoleukodystrophy white matten fatty acid abnormalities before the onset of demyelination. J Neural Sci 1992; 110:195-204.
    • (1992) J Neural Sci , vol.110 , pp. 195-204
    • Theda, C.1    Moser, A.B.2    Powers, J.M.3    Moser, H.W.4
  • 46
    • 0024507789 scopus 로고
    • Polyunsaturated fatty acid changes suggesting a new enzymatic defect in Zellweger syndrome
    • Martinez M. Polyunsaturated fatty acid changes suggesting a new enzymatic defect in Zellweger syndrome. Lipids 1989; 24:261-265.
    • (1989) Lipids , vol.24 , pp. 261-265
    • Martinez, M.1
  • 47
    • 0025039469 scopus 로고
    • Severe deficiency of docosahexaenoic acid in peroxisomal disorders: A defect of Δ-desaturation?
    • Martinez M. Severe deficiency of docosahexaenoic acid in peroxisomal disorders: a defect of Δ-desaturation? Neurology 1990; 40:1292-1298.
    • (1990) Neurology , vol.40 , pp. 1292-1298
    • Martinez, M.1
  • 48
    • 0026719737 scopus 로고
    • Severe changes in polyunsaturated fatty acids in the brain, liver, kidney, and retina in patients with peroxisomal disorders
    • Bazan N. (editor). New York: Plenum
    • Martinez M. Severe changes in polyunsaturated fatty acids in the brain, liver, kidney, and retina in patients with peroxisomal disorders. In: Neurobiology of essential fatty acids. Bazan N. (editor). New York: Plenum; 1992. pp. 347-359.
    • (1992) Neurobiology of Essential Fatty Acids , pp. 347-359
    • Martinez, M.1
  • 49
    • 0026715768 scopus 로고
    • Abnormal profiles of polyunsaturated fatty acids in the brain, liver, kidney and retina of patients with peroxisomal disorders
    • Martinez M. Abnormal profiles of polyunsaturated fatty acids in the brain, liver, kidney and retina of patients with peroxisomal disorders. Brain Res 1992; 583:171-182.
    • (1992) Brain Res , vol.583 , pp. 171-182
    • Martinez, M.1
  • 50
    • 0029584553 scopus 로고
    • Polyunsaturated fatty acids in the developing human brain, erythrocytes and plasma in peroxisomal disease: Therapeutic implications
    • Martinez M. Polyunsaturated fatty acids in the developing human brain, erythrocytes and plasma in peroxisomal disease: therapeutic implications. J Inherit Metab Dis 1995; 18 (Suppl. 1):61-75.
    • (1995) J Inherit Metab Dis , vol.18 , Issue.1 SUPPL. , pp. 61-75
    • Martinez, M.1
  • 51
    • 0028293574 scopus 로고
    • Blood polyunsaturated fatty acids in patients with peroxisomal disorders. A multicenter study
    • Martinez M, Mougan I, Roig M, Ballabriga A. Blood polyunsaturated fatty acids in patients with peroxisomal disorders. A multicenter study. Lipids 1994; 29:273-280.
    • (1994) Lipids , vol.29 , pp. 273-280
    • Martinez, M.1    Mougan, I.2    Roig, M.3    Ballabriga, A.4
  • 52
    • 0031004604 scopus 로고    scopus 로고
    • Evidence for the involvement of docosahexaenoic acid in cholinergic stimulated signal transduction at the synapse
    • Jones CR, Arai T, Rapoport SI. Evidence for the involvement of docosahexaenoic acid in cholinergic stimulated signal transduction at the synapse. Neurochem Res 1997; 22:663-670.
    • (1997) Neurochem Res , vol.22 , pp. 663-670
    • Jones, C.R.1    Arai, T.2    Rapoport, S.I.3
  • 53
    • 0022445141 scopus 로고
    • Biochemical and functional effects of prenatal and postnatal omega-3 fatty acid deficiency on retina and brain in rhesus monkeys
    • Neuringer M, Connor WE, Lin DS, Barstad L, Luck S. Biochemical and functional effects of prenatal and postnatal omega-3 fatty acid deficiency on retina and brain in rhesus monkeys. Proc Natl Acad Sci USA 1986; 83:4021-4025.
    • (1986) Proc Natl Acad Sci USA , vol.83 , pp. 4021-4025
    • Neuringer, M.1    Connor, W.E.2    Lin, D.S.3    Barstad, L.4    Luck, S.5
  • 54
    • 0026735539 scopus 로고
    • Treatment with docosahexaenoic acid favorably modifies the fatty acid composition of erythrocytes in peroxisomal patients
    • Coates PM, Tanaka K (editors). New York: Wiley-Liss
    • Martinez M. Treatment with docosahexaenoic acid favorably modifies the fatty acid composition of erythrocytes in peroxisomal patients. In: New developments in fatty acid oxidation. Coates PM, Tanaka K (editors). New York: Wiley-Liss, 1992. pp. 389-397.
    • (1992) New Developments in Fatty Acid Oxidation , pp. 389-397
    • Martinez, M.1
  • 55
    • 0027319874 scopus 로고
    • Docosahexaenoic acid - A new therapeutic approach to peroxisomal-disorder patients: Experience with two cases
    • Martinez M, Pineda M, Vidal R, Martin B. Docosahexaenoic acid - a new therapeutic approach to peroxisomal-disorder patients: experience with two cases. Neurology 1993; 43:1389-1397.
    • (1993) Neurology , vol.43 , pp. 1389-1397
    • Martinez, M.1    Pineda, M.2    Vidal, R.3    Martin, B.4
  • 56
    • 0032781202 scopus 로고    scopus 로고
    • Fatty acid composition of brain glycerophospholipids in peroxisomal disorders
    • Martinez M, Mougan I. Fatty acid composition of brain glycerophospholipids in peroxisomal disorders. Lipids 1999; 34:733-740. This paper reveals the fatty acid composition of plasmalogens in the brain of patients with peroxisomal disorders.
    • (1999) Lipids , vol.34 , pp. 733-740
    • Martinez, M.1    Mougan, I.2
  • 57
    • 0025841905 scopus 로고
    • The metabolism of 7,10,13,16,19 docosapentaenoic acid to 4,7,10,13,16,19-docosahexaenoic acid in rat liver is independent of a 4-desaturase
    • Voss A, Reinhart M, Sankarappa S, Sprecher H. The metabolism of 7,10,13,16,19 docosapentaenoic acid to 4,7,10,13,16,19-docosahexaenoic acid in rat liver is independent of a 4-desaturase. J Biol Chem 1991; 266:19995-20000.
    • (1991) J Biol Chem , vol.266 , pp. 19995-20000
    • Voss, A.1    Reinhart, M.2    Sankarappa, S.3    Sprecher, H.4
  • 58
    • 0033093771 scopus 로고    scopus 로고
    • An update on the pathways of polyunsaturated fatty acid metabolism
    • Sprecher H. An update on the pathways of polyunsaturated fatty acid metabolism. Curr Opin Clin Nutr Metab Care 1999; 2:135-138. An interesting review of the current concepts on the metabolism of long chain polyunsaturated fatty acids.
    • (1999) Curr Opin Clin Nutr Metab Care , vol.2 , pp. 135-138
    • Sprecher, H.1
  • 59
    • 0020400980 scopus 로고
    • Maladie de Refsum: Dix ans de régime pauvre en acide phytanique et phytol
    • Paris
    • Dry J, Pradalier A, Canny M. Maladie de Refsum: dix ans de régime pauvre en acide phytanique et phytol. Ann Med Interne (Paris) 1982; 133:483-487.
    • (1982) Ann Med Interne , vol.133 , pp. 483-487
    • Dry, J.1    Pradalier, A.2    Canny, M.3
  • 63
    • 0026742469 scopus 로고
    • Bone dysplasia associated with phytanic acid accumulation and deficient plasmalogen synthesis: A peroxisomal entity amenable to plasmapheresis
    • Smeitink JMA, Beemer FA, Speel M, Donckerwolcke RAMG, Jakobs C, Wanders RJA, et al. Bone dysplasia associated with phytanic acid accumulation and deficient plasmalogen synthesis: a peroxisomal entity amenable to plasmapheresis. J Inherit Metab Dis 1992; 15:377-380.
    • (1992) J Inherit Metab Dis , vol.15 , pp. 377-380
    • Smeitink, J.M.A.1    Beemer, F.A.2    Speel, M.3    Donckerwolcke, R.4    Jakobs, C.5    Wanders, R.J.A.6
  • 64
    • 0021961904 scopus 로고
    • Unsuccessful attempts to induce peroxisomes in two cases of Zellweger disease by treatment with clofibrate
    • Bjorkhem I, Blomstrand S, Glaumann H, Strandvik B. Unsuccessful attempts to induce peroxisomes in two cases of Zellweger disease by treatment with clofibrate. Pediatr Res 1985; 19:590-593.
    • (1985) Pediatr Res , vol.19 , pp. 590-593
    • Bjorkhem, I.1    Blomstrand, S.2    Glaumann, H.3    Strandvik, B.4
  • 65
    • 0015966787 scopus 로고
    • Some chemical aspects of human brain development. II. Phosphoglyceride fatty acids
    • Martinez M, Conde C, Ballabriga A. Some chemical aspects of human brain development. II. Phosphoglyceride fatty acids. Pediatr Res 1974; 8:91-102.
    • (1974) Pediatr Res , vol.8 , pp. 91-102
    • Martinez, M.1    Conde, C.2    Ballabriga, A.3
  • 66
    • 0026534480 scopus 로고
    • Tissue levels of polyunsaturated fatty acids during early human development
    • Martinez M. Tissue levels of polyunsaturated fatty acids during early human development. J Pediatr 1992; 120:S129-138.
    • (1992) J Pediatr , vol.120
    • Martinez, M.1
  • 67
    • 0018068702 scopus 로고
    • A chemical study on the development of the human forebrain and cerebellum during the brain 'growth spurt' period. I. Gangliosides and plasmalogens
    • Martinez M, Ballabriga A. A chemical study on the development of the human forebrain and cerebellum during the brain 'growth spurt' period. I. Gangliosides and plasmalogens. Brain Res 1978; 159:351-362.
    • (1978) Brain Res , vol.159 , pp. 351-362
    • Martinez, M.1    Ballabriga, A.2
  • 68
    • 0002472696 scopus 로고
    • Morphogenesis of the visual cortex in the preterm infant
    • Brazier MAB (editor). New York: Raven Press
    • Purpura DP. Morphogenesis of the visual cortex in the preterm infant. In: Growth and development of the brain. Brazier MAB (editor). New York: Raven Press; 1975. pp. 33-49.
    • (1975) Growth and Development of the Brain , pp. 33-49
    • Purpura, D.P.1
  • 69
    • 0020363004 scopus 로고
    • Myelin lipids in the developing cerebrum, cerebellum, and brain stem of normal and undernourished children
    • Martinez M. Myelin lipids in the developing cerebrum, cerebellum, and brain stem of normal and undernourished children. J Neurochem 1982; 39:1684-1692.
    • (1982) J Neurochem , vol.39 , pp. 1684-1692
    • Martinez, M.1
  • 70
    • 0031773487 scopus 로고    scopus 로고
    • Fatty acid composition of human brain phospholipids during normal development
    • Martinez M, Mougan I. Fatty acid composition of human brain phospholipids during normal development. J. Neurochem 1998; 71:2528-2533.
    • (1998) J. Neurochem , vol.71 , pp. 2528-2533
    • Martinez, M.1    Mougan, I.2
  • 71
    • 0003127498 scopus 로고
    • Vulnerable periods in developing brain
    • Davison AN, Dobbing J (editors). Edinburgh: Blackwell
    • Dobbing J. Vulnerable periods in developing brain. In: Applied Neurochemistry. Davison AN, Dobbing J (editors). Edinburgh: Blackwell 1968. pp. 287-316.
    • (1968) Applied Neurochemistry , pp. 287-316
    • Dobbing, J.1
  • 72
    • 0018193344 scopus 로고
    • Early postnatal starvation causes lasting hypomyelination
    • Wiggins RC, Fuller GN. Early postnatal starvation causes lasting hypomyelination. J Neurochem 1978; 30:1231-1237.
    • (1978) J Neurochem , vol.30 , pp. 1231-1237
    • Wiggins, R.C.1    Fuller, G.N.2
  • 73
    • 0029912369 scopus 로고    scopus 로고
    • Docosahexaenoic acid therapy in DHA-deficient patients with disorders of peroxisomal biogenesis
    • Martínez M. Docosahexaenoic acid therapy in DHA-deficient patients with disorders of peroxisomal biogenesis. Lipids 1996; 31:S145-152.
    • (1996) Lipids , vol.31
    • Martínez, M.1
  • 74
    • 0004496263 scopus 로고    scopus 로고
    • Docosahexaenoic acid ethyl ester as a treatment for patients with generalized peroxisomal disorders
    • Huang YS, Sinclair AJ (editors). Champaign, Illinois: AOCS Press
    • Martinez M. Docosahexaenoic acid ethyl ester as a treatment for patients with generalized peroxisomal disorders. In Lipids in infant nutrition. Huang YS, Sinclair AJ (editors). Champaign, Illinois: AOCS Press; 1998. pp. 29-38.
    • (1998) Lipids in Infant Nutrition , pp. 29-38
    • Martinez, M.1
  • 75
    • 0031869030 scopus 로고    scopus 로고
    • MRI evidence that docosahexaenoic acid ethyl ester improves myelination in generalized peroxisomal disorders
    • Martinez M, Vazquez E. MRI evidence that docosahexaenoic acid ethyl ester improves myelination in generalized peroxisomal disorders. Neurology 1998; 51:26-32.
    • (1998) Neurology , vol.51 , pp. 26-32
    • Martinez, M.1    Vazquez, E.2
  • 77
    • 0033964508 scopus 로고    scopus 로고
    • Therapeutical effects of docosahexaenoic acid in patients with generalized peroxisomal disorders
    • Martinez M, Vazquez E, García Silva MT, Manzanares J, Bertran JM, Castelló F, Mougan I. Therapeutical effects of docosahexaenoic acid in patients with generalized peroxisomal disorders. Am J Clin Nutr 2000; 71 (Suppl):376S-385S. This article summarizes the outcome of 7 years of DHA therapy in a small group of patients with generalized peroxisomal disorders, and gives some basic concepts on this new treatment.
    • (2000) Am J Clin Nutr , vol.71 , Issue.SUPPL.
    • Martinez, M.1    Vazquez, E.2    García Silva, M.T.3    Manzanares, J.4    Bertran, J.M.5    Castelló, F.6    Mougan, I.7


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.