메뉴 건너뛰기




Volumn 804, Issue , 1996, Pages 450-460

Metabolic aspects of peroxisomal disorders

Author keywords

[No Author keywords available]

Indexed keywords

FATTY ACID; PHOSPHOLIPID; PHYTANIC ACID; PIPECOLIC ACID;

EID: 0030450007     PISSN: 00778923     EISSN: None     Source Type: Book Series    
DOI: 10.1111/j.1749-6632.1996.tb18636.x     Document Type: Conference Paper
Times cited : (17)

References (38)
  • 2
    • 0020345656 scopus 로고
    • Cerebro-hepato-renal (Zellweger) syndrome and neonatal adrenoleukodystrophy: Similarities in phenotype and accumulation of very long chain fatty acids
    • BROWN, F. R., A. J. MCADAMS, J. W. CUMMINS, R. KONKOL, I. SINGH, A. E. MOSER & H. W. MOSER. 1982. Cerebro-hepato-renal (Zellweger) syndrome and neonatal adrenoleukodystrophy: Similarities in phenotype and accumulation of very long chain fatty acids. John Hopkins Med. J. 151: 344-361.
    • (1982) John Hopkins Med. J. , vol.151 , pp. 344-361
    • Brown, F.R.1    Mcadams, A.J.2    Cummins, J.W.3    Konkol, R.4    Singh, I.5    Moser, A.E.6    Moser, H.W.7
  • 5
    • 0028068292 scopus 로고
    • Peroxisomal lipid metabolism
    • REDDY, J. K. & G. P. MANNAERTS. 1994. Peroxisomal lipid metabolism. Annu.Rev.Nutr. 14: 343-370.
    • (1994) Annu.Rev.Nutr. , vol.14 , pp. 343-370
    • Reddy, J.K.1    Mannaerts, G.P.2
  • 6
    • 0000228425 scopus 로고
    • Disorders of peroxisome biogenesis
    • C. R. Scriver, A. L. Beadet, W. S. Sly & D. Valle, Eds. McGraw-Hill, New York
    • LAZAROW, P. B. & H. W. MOSER. 1995. Disorders of peroxisome biogenesis. In The Metabolic and Molecular Bases of Inherited Disease C. R. Scriver, A. L. Beadet, W. S. Sly & D. Valle, Eds. Vol. 5: 2287-3324. McGraw-Hill, New York.
    • (1995) The Metabolic and Molecular Bases of Inherited Disease , vol.5 , pp. 2287-3324
    • Lazarow, P.B.1    Moser, H.W.2
  • 7
    • 0027278174 scopus 로고
    • Studies on phytanic acid alpha-oxidation in rat liver and cultured skin fibroblasts
    • WANDERS, R. J. A. & C. W. T. VAN ROERMUND. 1993. Studies on phytanic acid alpha-oxidation in rat liver and cultured skin fibroblasts. Biochim.Biophys. Acta 1167: 345-350.
    • (1993) Biochim.Biophys. Acta , vol.1167 , pp. 345-350
    • Wanders, R.J.A.1    Van Roermund, C.W.T.2
  • 8
    • 0026528902 scopus 로고
    • Pristanic acid and phytanic acid in plasma from patients with peroxisomal disorders: Stable isotope dilution analysis with electron capture negative ion mass fragmentography
    • TEN BRINK, H. J., F. STELLAARD, C. M. M. VAN DE HEUVEL, D. S. M. SCHOR, R. J. A. WANDERS & C. JAKOBS. 1992. Pristanic acid and phytanic acid in plasma from patients with peroxisomal disorders: Stable isotope dilution analysis with electron capture negative ion mass fragmentography. J.Lipid Res. 33: 31-37.
    • (1992) J.Lipid Res. , vol.33 , pp. 31-37
    • Ten Brink, H.J.1    Stellaard, F.2    Van De Heuvel, C.M.M.3    Schor, D.S.M.4    Wanders, R.J.A.5    Jakobs, C.6
  • 9
    • 0028102575 scopus 로고
    • 2-Hydroxyphytanic acid oxidase activity in rat and human liver and its deficiency in the Zellweger syndrome
    • WANDERS, R. J. A., C. W. T. VAN ROERMUND, D. S. M. SCHOR, H. J. TEN BRINK & C. JACOBS. 1994. 2-Hydroxyphytanic acid oxidase activity in rat and human liver and its deficiency in the Zellweger syndrome. Biochim.Biophys.Acta 1227: 177-182.
    • (1994) Biochim.Biophys.Acta , vol.1227 , pp. 177-182
    • Wanders, R.J.A.1    Van Roermund, C.W.T.2    Schor, D.S.M.3    Ten Brink, H.J.4    Jacobs, C.5
  • 10
    • 0027270301 scopus 로고
    • Phytanic acid alpha-oxidation: Differential subcellular localization in rat and human tissues and its inhibition by Nycodenz
    • SINGH, I., K. PAHAN, G. S. DHAUNSI, O. LAZO & P. OZAND. 1993. Phytanic acid alpha-oxidation: differential subcellular localization in rat and human tissues and its inhibition by Nycodenz. J.Biol.Chem. 268: 9972-9979.
    • (1993) J.Biol.Chem. , vol.268 , pp. 9972-9979
    • Singh, I.1    Pahan, K.2    Dhaunsi, G.S.3    Lazo, O.4    Ozand, P.5
  • 11
    • 0027311182 scopus 로고
    • Formic acid is a product of the alpha-oxidation of fatty acids by human skin fibroblasts: Deficiency of formic acid production in peroxisome deficient fibroblasts
    • POULOS, A., P. SHARP, H. SINGH, D. W. JOHNSON, W. F. CAREY & C. EASTON. 1993. Formic acid is a product of the alpha-oxidation of fatty acids by human skin fibroblasts: Deficiency of formic acid production in peroxisome deficient fibroblasts. Biochem.J. 292: 457-461.
    • (1993) Biochem.J. , vol.292 , pp. 457-461
    • Poulos, A.1    Sharp, P.2    Singh, H.3    Johnson, D.W.4    Carey, W.F.5    Easton, C.6
  • 12
    • 0027993403 scopus 로고
    • Aminotriazole is a potent inhibitor of alpha-oxidation of 3-methyl-substituted fatty acids in rat liver
    • CASTEELS, M., K. CROES, P. P. VAN VELDHOVEN & G. P. MANNAERTS. 1994. Aminotriazole is a potent inhibitor of alpha-oxidation of 3-methyl-substituted fatty acids in rat liver. Biochem. Pharmacol. 48: 1973-1975.
    • (1994) Biochem. Pharmacol. , vol.48 , pp. 1973-1975
    • Casteels, M.1    Croes, K.2    Van Veldhoven, P.P.3    Mannaerts, G.P.4
  • 14
    • 0027289172 scopus 로고
    • Identification of phytanoyl-CoA ligase as a distinct acyl-CoA ligase in peroxisomes from cultured skin fibroblasts
    • PAHAN, K., J. COFER, P. BALIGA & I. SINGH. 1993. Identification of phytanoyl-CoA ligase as a distinct acyl-CoA ligase in peroxisomes from cultured skin fibroblasts. FEBS Lett. 322: 101-104.
    • (1993) FEBS Lett. , vol.322 , pp. 101-104
    • Pahan, K.1    Cofer, J.2    Baliga, P.3    Singh, I.4
  • 15
    • 0028883208 scopus 로고
    • Substrate specificity of rat liver mitochondrial carnitine palmitoyl-transferase I: Evidence against a-oxidation of phytanic acid in rat liver mitochondria
    • SINGH, H. & A. POULOS. 1995. Substrate specificity of rat liver mitochondrial carnitine palmitoyl-transferase I: Evidence against a-oxidation of phytanic acid in rat liver mitochondria. FEBS Lett. 359: 179-183.
    • (1995) FEBS Lett. , vol.359 , pp. 179-183
    • Singh, H.1    Poulos, A.2
  • 16
    • 0028027674 scopus 로고
    • Phytanic acid must be activated to phytanoyl-CoA prior to its α-oxidation in rat liver peroxisomes
    • WATKINS, P.A., A.E. HOWARD & S.J. MIHALIK. 1994. Phytanic acid must be activated to phytanoyl-CoA prior to its α-oxidation in rat liver peroxisomes. Biochim. Biophys. Acta 1214: 288-294.
    • (1994) Biochim. Biophys. Acta , vol.1214 , pp. 288-294
    • Watkins, P.A.1    Howard, A.E.2    Mihalik, S.J.3
  • 17
    • 0025039469 scopus 로고
    • Severe deficiency of docosahexaenoic acid in peroxisomal disorders: A defect in 4-desaturase
    • MARTINEZ, M. 1990. Severe deficiency of docosahexaenoic acid in peroxisomal disorders: a defect in 4-desaturase. Neurology 40: 1292-1298.
    • (1990) Neurology , vol.40 , pp. 1292-1298
    • Martinez, M.1
  • 18
    • 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., M. REINHART, S. SANKARAPPA & H. SPRECHER. 1991. 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. 266: 19995-20000.
    • (1991) J.Biol.Chem. , vol.266 , pp. 19995-20000
    • Voss, A.1    Reinhart, M.2    Sankarappa, S.3    Sprecher, H.4
  • 20
    • 0028944018 scopus 로고
    • Cholesterol biosynthesis in dermal fibroblasts from patients with metabolic disorders of peroxisomal origin
    • MALLE, E., K. OETTL, W. SATTLER, G. HOEFLER & G. M. KOSTNER. 1995. Cholesterol biosynthesis in dermal fibroblasts from patients with metabolic disorders of peroxisomal origin. Eur. J. Clin. Invest. 25: 59-67.
    • (1995) Eur. J. Clin. Invest. , vol.25 , pp. 59-67
    • Malle, E.1    Oettl, K.2    Sattler, W.3    Hoefler, G.4    Kostner, G.M.5
  • 21
    • 0023878166 scopus 로고
    • A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase
    • POLL-THE, B. T., F. ROELS, H. OGIER & H. SCOTTO. 1988. A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase. Am. J. Hum. Genet. 42: 422-434.
    • (1988) Am. J. Hum. Genet. , vol.42 , pp. 422-434
    • Poll-The, B.T.1    Roels, F.2    Ogier, H.3    Scotto, H.4
  • 25
    • 0025047621 scopus 로고
    • Peroxisomal disorders: Complementation analysis using β-oxidation of very long chain fatty acids
    • MCGUINESS, M. C., A. B. MOSER, H. W. MOSER & P. B. WATKINS. 1990. Peroxisomal disorders: Complementation analysis using β-oxidation of very long chain fatty acids. Biochem. Biophys. Res. Commun. 172: 364-369.
    • (1990) Biochem. Biophys. Res. Commun. , vol.172 , pp. 364-369
    • Mcguiness, M.C.1    Moser, A.B.2    Moser, H.W.3    Watkins, P.B.4
  • 29
    • 0028332245 scopus 로고
    • Differential protein import deficiencies in human peroxisome assembly disorders
    • MOTLEY, A., E. HETTEMA, B. DISTEL & H. TABAK. 1994. Differential protein import deficiencies in human peroxisome assembly disorders. J. Cell. Biol. 125: 755-767.
    • (1994) J. Cell. Biol. , vol.125 , pp. 755-767
    • Motley, A.1    Hettema, E.2    Distel, B.3    Tabak, H.4
  • 30
    • 0028053931 scopus 로고
    • PAS7 encodes a novel yeast member of the WD-40 protein family essential for import of 3-oxoacyl-CoA thiolase, a PTS2-containing protein, into peroxisomes
    • MARZIOCH, M., R. ERDMANN, M. VEENHUIS & W. H. KUNAU. 1994. PAS7 encodes a novel yeast member of the WD-40 protein family essential for import of 3-oxoacyl-CoA thiolase, a PTS2-containing protein, into peroxisomes. EMBO J. 13: 4908-4917.
    • (1994) EMBO J. , vol.13 , pp. 4908-4917
    • Marzioch, M.1    Erdmann, R.2    Veenhuis, M.3    Kunau, W.H.4
  • 33
    • 0023782245 scopus 로고
    • A possible role for plasmalogens in protecting animal cells against photosensitized killing
    • Zoeller, R.A., O. H. Morand & C. R .H. Raetz. 1988. A possible role for plasmalogens in protecting animal cells against photosensitized killing. J. Biol. Chem. 263: 11590-11596.
    • (1988) J. Biol. Chem. , vol.263 , pp. 11590-11596
    • Zoeller, R.A.1    Morand, O.H.2    Raetz, C.R.H.3
  • 34
    • 8044237962 scopus 로고    scopus 로고
    • Plasmalogens and oxidative stress: Evidence against a major role of plasmalogens in protection against the superoxide anion radical
    • In press
    • JANSEN, G. A. & R. J. A. WANDERS. 1996. Plasmalogens and oxidative stress: Evidence against a major role of plasmalogens in protection against the superoxide anion radical. J. Inher. Metab. Dis. In press.
    • (1996) J. Inher. Metab. Dis.
    • Jansen, G.A.1    Wanders, R.J.A.2
  • 35
    • 0028978668 scopus 로고
    • Phytanic acid β-oxidation in rat liver peroxisomes: Production of α-hydroxyphytanoyl-CoA and formate is enhanced by dioxygenase cofactors
    • MIHALIK, S. J., A. N. RAINVILLE & P. A. WATKINS. 1995. Phytanic acid β-oxidation in rat liver peroxisomes: Production of α-hydroxyphytanoyl-CoA and formate is enhanced by dioxygenase cofactors. Eur. J. Biochem. 232: 545-551.
    • (1995) Eur. J. Biochem. , vol.232 , pp. 545-551
    • Mihalik, S.J.1    Rainville, A.N.2    Watkins, P.A.3
  • 38
    • 7844237717 scopus 로고    scopus 로고
    • Phytanoyl-CoA hydroxylase deficiency: Discovery of the long sought enzyme defect in Refsum disease and a possible therapy
    • Submitted for publication
    • JANSEN, G. A., R. J. A. WANDERS, C. J. DE GROOT, C. JAKOBS, H. W. MOSER, P. A. WATKINS & S. J. MIHALIK. 1996. Phytanoyl-CoA hydroxylase deficiency: Discovery of the long sought enzyme defect in Refsum disease and a possible therapy. N. Engl. J. Med. Submitted for publication.
    • (1996) N. Engl. J. Med.
    • Jansen, G.A.1    Wanders, R.J.A.2    De Groot, C.J.3    Jakobs, C.4    Moser, H.W.5    Watkins, P.A.6    Mihalik, S.J.7


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.