-
1
-
-
0027322675
-
Peroxisomal disorders: Neurodevelopmental and biochemical aspects
-
Brown F R III, Voigt R, Singh A K, Singh I. Peroxisomal disorders: Neurodevelopmental and biochemical aspects. Am J Dis Child. 147:1993;617-626.
-
(1993)
Am J Dis Child
, vol.147
, pp. 617-626
-
-
Brown F.R. III1
Voigt, R.2
Singh, A.K.3
Singh, I.4
-
2
-
-
0023902953
-
Molecular analysis of peroxisomal β-oxidation enzymes in infants with Zellweger syndrome and Zellweger-like syndrome: Further heterogenity of the peroxisomal disorders
-
Suzuki Y, Shimozawa N, Orii T, Igarashi N, Kono N, Hashimoto T. Molecular analysis of peroxisomal β-oxidation enzymes in infants with Zellweger syndrome and Zellweger-like syndrome: Further heterogenity of the peroxisomal disorders. Clin Chim Acta. 172:1988;65-76.
-
(1988)
Clin Chim Acta
, vol.172
, pp. 65-76
-
-
Suzuki, Y.1
Shimozawa, N.2
Orii, T.3
Igarashi, N.4
Kono, N.5
Hashimoto, T.6
-
3
-
-
0027234217
-
Pseudo infantile Refsum disease: Catalase-negative peroxisomal particles with partial plasmalogen synthesis and oxidation of fatty acids
-
Aubourg P, Kremser K, Roland M O, Rocchiccioli F, Singh I. Pseudo infantile Refsum disease: Catalase-negative peroxisomal particles with partial plasmalogen synthesis and oxidation of fatty acids. Pediatr Res. 34:1993;270-276.
-
(1993)
Pediatr Res
, vol.34
, pp. 270-276
-
-
Aubourg, P.1
Kremser, K.2
Roland, M.O.3
Rocchiccioli, F.4
Singh, I.5
-
4
-
-
0020345656
-
Cerebro-hepato-renal (Zellweger) syndrome and neonatal adrenoleukodystrophy: Similarities in phenotype and accumulation of very long chain fatty acids
-
Brown F R III, McAdams A, Cummins J, Konkel R, Singh I, Moser A, Moser H. Cerebro-hepato-renal (Zellweger) syndrome and neonatal adrenoleukodystrophy: Similarities in phenotype and accumulation of very long chain fatty acids. Johns Hopkins Med. J. 151:1982;344-351.
-
(1982)
Johns Hopkins Med. J
, vol.151
, pp. 344-351
-
-
Brown F.R. III1
McAdams, A.2
Cummins, J.3
Konkel, R.4
Singh, I.5
Moser, A.6
Moser, H.7
-
5
-
-
0015795115
-
Synthesis of cerebronic acid from lignoceric acid by rat brain preparation. Some properties and distribution of the hydroxylation system
-
Hoshi M, Kishimoto Y. Synthesis of cerebronic acid from lignoceric acid by rat brain preparation. Some properties and distribution of the hydroxylation system. J Biol Chem. 248:1973;4123-4130.
-
(1973)
J Biol Chem
, vol.248
, pp. 4123-4130
-
-
Hoshi, M.1
Kishimoto, Y.2
-
6
-
-
0026479078
-
Phytanic acid a-oxidation in human cultured skin fibroblasts
-
Singh I, Lazo O, Pahan K, Singh A K. Phytanic acid a-oxidation in human cultured skin fibroblasts. Biochim Biophys Acta. 1180:1992;221-224.
-
(1992)
Biochim Biophys Acta
, vol.1180
, pp. 221-224
-
-
Singh, I.1
Lazo, O.2
Pahan, K.3
Singh, A.K.4
-
7
-
-
0022977285
-
Acyl-CoA: Dihydroxyacetone phosphate acyltransferase in human skin fibroblasts: Study of its properties using a new assay method
-
Schutgens R BH, Romeyn G J, Of man R, van den Bosch H, Tager J M, Wanders R J. Acyl-CoA: dihydroxyacetone phosphate acyltransferase in human skin fibroblasts: Study of its properties using a new assay method. Biochim Biophys Acta. 879:1986;286-291.
-
(1986)
Biochim Biophys Acta
, vol.879
, pp. 286-291
-
-
Schutgens, R.B.H.1
Romeyn, G.J.2
Of Man, R.3
Van Den Bosch, H.4
Tager, J.M.5
Wanders, R.J.6
-
8
-
-
0021161650
-
Activity of peroxisomal enzymes and intracellular distribution of catalase in Zellweger syndrome
-
Wanders R J, Kos M, Roest B, Meijer A J, Schrakamp G, Heymans H S, Tegelaers W H, van den Bosch H, Schutgens R B, Tager J M. Activity of peroxisomal enzymes and intracellular distribution of catalase in Zellweger syndrome. Biochem Biophys Res Commun. 123:1984;1054-1061.
-
(1984)
Biochem Biophys Res Commun
, vol.123
, pp. 1054-1061
-
-
Wanders, R.J.1
Kos, M.2
Roest, B.3
Meijer, A.J.4
Schrakamp, G.5
Heymans, H.S.6
Tegelaers, W.H.7
Van Den Bosch, H.8
Schutgens, R.B.9
Tager, J.M.10
-
9
-
-
0021265709
-
The cerebrohepatorenal (Zellweger) syndrome increased levels and impaired degradation of very-long-chain fatty acids and their use in prenatal diagnosis
-
Moser A RE, Singh I, Brown F R III, Solish G I, Kelley R I, Benke P J, Moser H W. The cerebrohepatorenal (Zellweger) syndrome increased levels and impaired degradation of very-long-chain fatty acids and their use in prenatal diagnosis. N Engl J Med. 310:1984;1141-1146.
-
(1984)
N Engl J Med
, vol.310
, pp. 1141-1146
-
-
Moser, A.R.E.1
Singh, I.2
Brown F.R. III3
Solish, G.I.4
Kelley, R.I.5
Benke, P.J.6
Moser, H.W.7
-
10
-
-
0001112876
-
Peroxisomal lignoceroyl-CoA ligase deficiency in adrenoleukodystrophy and adrenomyeloneuropathy
-
Lazo O, Contreras M, Hashmi M, Stanley W, Irazu C, Singh I. Peroxisomal lignoceroyl-CoA ligase deficiency in adrenoleukodystrophy and adrenomyeloneuropathy. Proc Natl Acad Sci USA. 85:1988;7647-7651.
-
(1988)
Proc Natl Acad Sci USA
, vol.85
, pp. 7647-7651
-
-
Lazo, O.1
Contreras, M.2
Hashmi, M.3
Stanley, W.4
Irazu, C.5
Singh, I.6
-
12
-
-
0023932509
-
Peroxisomal membrane ghosts in Zellweger syndrome-aberrant organelle assembly
-
Santos M, Imanaka T, Shio H, Small G, Lazarow P. Peroxisomal membrane ghosts in Zellweger syndrome-aberrant organelle assembly. Science. 239:1988;1536-1538.
-
(1988)
Science
, vol.239
, pp. 1536-1538
-
-
Santos, M.1
Imanaka, T.2
Shio, H.3
Small, G.4
Lazarow, P.5
-
13
-
-
0023158322
-
A new dietary therapy for adrenoleukodystrophy: Biochemical and preliminary clinical result in 36 patients
-
Moser A B, Borel J, Odone A, Naidu S, Cornblath D, Sanders D B, Moser H W. A new dietary therapy for adrenoleukodystrophy: Biochemical and preliminary clinical result in 36 patients. Ann Neurol. 21:1987;240-249.
-
(1987)
Ann Neurol
, vol.21
, pp. 240-249
-
-
Moser, A.B.1
Borel, J.2
Odone, A.3
Naidu, S.4
Cornblath, D.5
Sanders, D.B.6
Moser, H.W.7
-
14
-
-
0022395527
-
A milder variant of Zellweger syndrome
-
Barth P, Schutgens B K, Bakkeren J, Dingemans K, Heymans H, Douwes A, Vander Klerian Moorsel J. A milder variant of Zellweger syndrome. Eur J Pediatr. 144:1985;338-342.
-
(1985)
Eur J Pediatr
, vol.144
, pp. 338-342
-
-
Barth, P.1
Schutgens, B.K.2
Bakkeren, J.3
Dingemans, K.4
Heymans, H.5
Douwes, A.6
Vander Klerian Moorsel, J.7
-
15
-
-
0022573511
-
Longterm survival of a patient with the cerebro-hepato-renal (Zellweger) syndrome
-
Bleeker-Wagemakers E, Oorthugs J, Wanders R, Schutgens R. Longterm survival of a patient with the cerebro-hepato-renal (Zellweger) syndrome. Clin Genet. 29:1990;160-164.
-
(1990)
Clin Genet
, vol.29
, pp. 160-164
-
-
Bleeker-Wagemakers, E.1
Oorthugs, J.2
Wanders, R.3
Schutgens, R.4
-
16
-
-
0025569547
-
Peroxisomal disorders
-
Naidu S, Moser H. Peroxisomal disorders. Int Pediatr. 5:1990;125-133.
-
(1990)
Int Pediatr
, vol.5
, pp. 125-133
-
-
Naidu, S.1
Moser, H.2
-
17
-
-
0022480922
-
Neonatal adrenoleukodystrophy: New cases, biochemical studies, and differentiation from Zellweger and related peroxisomal polydystrophy syndromes
-
Kelley R1, Datta N S, Dobyns W B, Hajra A, Moser A, Nuetzel M, Zackai E, Moser H. Neonatal adrenoleukodystrophy: New cases, biochemical studies, and differentiation from Zellweger and related peroxisomal polydystrophy syndromes. Am J Med Genet. 23:1986;869-901.
-
(1986)
Am J Med Genet
, vol.23
, pp. 869-901
-
-
Kelley, R.L.1
Datta, N.S.2
Dobyns, W.B.3
Hajra, A.4
Moser, A.5
Nuetzel, M.6
Zackai, E.7
Moser, H.8
-
18
-
-
0011078306
-
Neonatal adrenoleukodystrophy with prolonged survival
-
Noetzel M, Clark H, Moser H. Neonatal adrenoleukodystrophy with prolonged survival. Ann Neurol. 4:1983;379-380.
-
(1983)
Ann Neurol
, vol.4
, pp. 379-380
-
-
Noetzel, M.1
Clark, H.2
Moser, H.3
-
19
-
-
0023582841
-
Infantile Refsum disease: An inherited peroxisomal disorder: Comparison with Zellweger syndrome and neonatal adrenoleukodystrophy
-
Poll-The B T, Saudubray J M, Ogier H. Infantile Refsum disease: An inherited peroxisomal disorder: Comparison with Zellweger syndrome and neonatal adrenoleukodystrophy. Eur J Pediatr. 146:1987;477-483.
-
(1987)
Eur J Pediatr
, vol.146
, pp. 477-483
-
-
Poll-The, B.T.1
Saudubray, J.M.2
Ogier, H.3
-
20
-
-
0022163730
-
Biogenesis of peroxisomes. A review
-
Lazarow P, Fujiki Y. Biogenesis of peroxisomes. A review. J Cell Biol. 1:1985;489-530.
-
(1985)
J Cell Biol
, vol.1
, pp. 489-530
-
-
Lazarow, P.1
Fujiki, Y.2
-
21
-
-
0027333416
-
Protein import into peroxisomes and biogenesis of the organelle. A review
-
Subramani S. Protein import into peroxisomes and biogenesis of the organelle. A review. J Cell Biol. 9:1993;445-478.
-
(1993)
J Cell Biol
, vol.9
, pp. 445-478
-
-
Subramani, S.1
-
22
-
-
0023541627
-
Translocation of acyl-CoA oxidase into peroxisomes requires ATP hydrolysis but not a membrane potential
-
Imanaka T, Small G, Lazarow P. Translocation of acyl-CoA oxidase into peroxisomes requires ATP hydrolysis but not a membrane potential. J Cell Biol. 105:1987;2915-2922.
-
(1987)
J Cell Biol
, vol.105
, pp. 2915-2922
-
-
Imanaka, T.1
Small, G.2
Lazarow, P.3
-
23
-
-
0027178239
-
Import of firefly luciferase into mammalian peroxisomes in vivo requires nucleotide triphosphatase
-
Soto U, Pepperkok R, Ansorge W, Just W. Import of firefly luciferase into mammalian peroxisomes in vivo requires nucleotide triphosphatase. J Cell Biol. 205:1993;66-75.
-
(1993)
J Cell Biol
, vol.205
, pp. 66-75
-
-
Soto, U.1
Pepperkok, R.2
Ansorge, W.3
Just, W.4
-
24
-
-
0027448017
-
Cytosol-dependent peroxisomal protein import in a permealized cell system
-
Wendland M, Subramani S. Cytosol-dependent peroxisomal protein import in a permealized cell system. J Cell Biol. 120:1993;675-685.
-
(1993)
J Cell Biol
, vol.120
, pp. 675-685
-
-
Wendland, M.1
Subramani, S.2
-
25
-
-
0028242601
-
Involvement of 70kDa heat shock proteins in peroxisomal import
-
Walton P, Wendland M, Subramani S, Rachubinski R, Welch W. Involvement of 70kDa heat shock proteins in peroxisomal import. J Cell Biol. 125:1994;1037-1046.
-
(1994)
J Cell Biol
, vol.125
, pp. 1037-1046
-
-
Walton, P.1
Wendland, M.2
Subramani, S.3
Rachubinski, R.4
Welch, W.5
-
26
-
-
0026326482
-
Amino-terminal presequence of the precursor of the peroxisomal 3-ketoacyl-CoA thiolase is a cleavable signal peptide for peroxisomal targeting
-
Osumi T, Tsukamoto T, Hata S, Yokota S, Miura S, Fujiki Y, Hijikata M, Miyazawa S, Hashimoto T. Amino-terminal presequence of the precursor of the peroxisomal 3-ketoacyl-CoA thiolase is a cleavable signal peptide for peroxisomal targeting. Biochem Biophys Res Commun. 181:1991;947-954.
-
(1991)
Biochem Biophys Res Commun
, vol.181
, pp. 947-954
-
-
Osumi, T.1
Tsukamoto, T.2
Hata, S.3
Yokota, S.4
Miura, S.5
Fujiki, Y.6
Hijikata, M.7
Miyazawa, S.8
Hashimoto, T.9
-
27
-
-
0025737231
-
Rhizomelic chondrodysplasia punctata: Biochemical studies of peroxisomes isolated from cultured skin fibroblasts
-
Singh I, Lazo O, Contreras M, Stanley W, Hashimoto T. Rhizomelic chondrodysplasia punctata: Biochemical studies of peroxisomes isolated from cultured skin fibroblasts. Arch Biochem Biophys. 286:1991;277-283.
-
(1991)
Arch Biochem Biophys
, vol.286
, pp. 277-283
-
-
Singh, I.1
Lazo, O.2
Contreras, M.3
Stanley, W.4
Hashimoto, T.5
-
28
-
-
0025941962
-
A novel, cleavable peroxisomal targeting signal at the amino-terminus of the rat 3-ketoacyl-CoA thiolase
-
Swinkels B W, Gould S J, Bodnar A G, Rachubinski R A, Subramani S. A novel, cleavable peroxisomal targeting signal at the amino-terminus of the rat 3-ketoacyl-CoA thiolase. EMBO J. 10:1991;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
-
29
-
-
0028149887
-
Peroxisomal biogenesis: Multiple pathways of protein import
-
Purdue P E, Lazarow P B. Peroxisomal biogenesis: Multiple pathways of protein import. J Biol Chem. 269:1994;30065-30068.
-
(1994)
J Biol Chem
, vol.269
, pp. 30065-30068
-
-
Purdue, P.E.1
Lazarow, P.B.2
-
30
-
-
0027731109
-
Three peroxisome protein packaging pathways suggested by selective permeanilization of yeast mutant defective in peroxisome biogenesis
-
Zhang J W, Luckey C, Lazarow P B. Three peroxisome protein packaging pathways suggested by selective permeanilization of yeast mutant defective in peroxisome biogenesis. Mol Biol Cell. 4:1993;1350-1359.
-
(1993)
Mol Biol Cell
, vol.4
, pp. 1350-1359
-
-
Zhang, J.W.1
Luckey, C.2
Lazarow, P.B.3
-
31
-
-
0027451869
-
Alterations in peroxisomal function in ischemia-reperfusion injury of rat kidney
-
Gulati S, Ainol L, Orak J, Singh A K, Singh I. Alterations in peroxisomal function in ischemia-reperfusion injury of rat kidney. Biochim Biophys Acta. 1182:1993;291-298.
-
(1993)
Biochim Biophys Acta
, vol.1182
, pp. 291-298
-
-
Gulati, S.1
Ainol, L.2
Orak, J.3
Singh, A.K.4
Singh, I.5
|