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Year Number of Results
1987 1
1989 2
1996 1
1997 1
1998 2
1999 1
2000 2
2001 4
2003 2
2004 1
2012 2
2017 1
2018 1
2023 0

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21 results

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Page 1
Neonatal metabolic myopathies.
Tein I. Tein I. Semin Perinatol. 1999 Apr;23(2):125-51. doi: 10.1016/s0146-0005(99)80046-9. Semin Perinatol. 1999. PMID: 10331465 Review.
Disorders of glycogen, lipid, or mitochondrial metabolism may cause three main clinical syndromes in muscle, namely, (1) progressive weakness with hypotonia (e.g., acid maltase, debrancher enzyme, and brancher enzyme deficiencies among the glycogenoses; carnitine uptake an …
Disorders of glycogen, lipid, or mitochondrial metabolism may cause three main clinical syndromes in muscle, namely, (1) progressive weaknes …
Inborn Errors of Bile Acid Metabolism.
Heubi JE, Setchell KDR, Bove KE. Heubi JE, et al. Clin Liver Dis. 2018 Nov;22(4):671-687. doi: 10.1016/j.cld.2018.06.006. Epub 2018 Aug 22. Clin Liver Dis. 2018. PMID: 30266156 Review.
Peroxisomal beta-oxidation and steatohepatitis.
Rao MS, Reddy JK. Rao MS, et al. Semin Liver Dis. 2001;21(1):43-55. doi: 10.1055/s-2001-12928. Semin Liver Dis. 2001. PMID: 11296696 Review.
Evidence from mice deficient in PPAR alpha (PPAR alpha-/-), deficient in peroxisomal fatty acyl-CoA oxidase (AOX-/-), the first enzyme of the classical beta-oxidation system, and deficient in both PPAR alpha and AOX (PPAR alpha-/-AOX-/-) …
Evidence from mice deficient in PPAR alpha (PPAR alpha-/-), deficient in peroxisomal fatty acyl-CoA oxidase …
Clinical and Laboratory Diagnosis of Peroxisomal Disorders.
Wanders RJ, Klouwer FC, Ferdinandusse S, Waterham HR, Poll-Thé BT. Wanders RJ, et al. Methods Mol Biol. 2017;1595:329-342. doi: 10.1007/978-1-4939-6937-1_30. Methods Mol Biol. 2017. PMID: 28409475 Review.
Peroxisomal beta-oxidation and peroxisome proliferator-activated receptor alpha: an adaptive metabolic system.
Reddy JK, Hashimoto T. Reddy JK, et al. Annu Rev Nutr. 2001;21:193-230. doi: 10.1146/annurev.nutr.21.1.193. Annu Rev Nutr. 2001. PMID: 11375435 Review.
The peroxisomal beta-oxidation system consists of (a) a classical peroxisome proliferator-inducible pathway capable of catalyzing straight-chain acyl-CoAs by fatty acyl-CoA oxidase, L-bifunctional protein, and thiolase, and (b) a second noninducible pa …
The peroxisomal beta-oxidation system consists of (a) a classical peroxisome proliferator-inducible pathway capable of catalyzing straight-c …
Clinical consequences of defects in peroxisomal beta-oxidation.
Clayton PT. Clayton PT. Biochem Soc Trans. 2001 May;29(Pt 2):298-305. doi: 10.1042/0300-5127:0290298. Biochem Soc Trans. 2001. PMID: 11356171 Review.
The disorders of peroxisomal beta-oxidation, which have been well characterised at the molecular level, include defects of acyl-CoA oxidase, defects of the D-bifunctional protein (D-BP) (including specific defects of its enoyl-CoA hydratase and D-3-hyd …
The disorders of peroxisomal beta-oxidation, which have been well characterised at the molecular level, include defects of acyl-Co …
Biogenesis of peroxisomes in fetal liver.
Espeel M, Depreter M, Nardacci R, D'Herde K, Kerckaert I, Stefanini S, Roels F. Espeel M, et al. Microsc Res Tech. 1997 Dec 1;39(5):453-66. doi: 10.1002/(SICI)1097-0029(19971201)39:5<453::AID-JEMT8>3.0.CO;2-H. Microsc Res Tech. 1997. PMID: 9408912 Review.
The present article gives an overview of microscopic studies and recent unpublished results dealing with peroxisome biogenesis in mammalian fetal liver and presents data on peroxisomes in oocytes. Cytochemical (catalase and D-aminoacid oxidase activity) and immunocytochemi …
The present article gives an overview of microscopic studies and recent unpublished results dealing with peroxisome biogenesis in mammalian …
Prenatal and perinatal diagnosis of peroxisomal disorders.
Schutgens RB, Schrakamp G, Wanders RJ, Heymans HS, Tager JM, van den Bosch H. Schutgens RB, et al. J Inherit Metab Dis. 1989;12 Suppl 1:118-34. doi: 10.1007/BF01799291. J Inherit Metab Dis. 1989. PMID: 2509803 Review.
Peroxisomal disorders, a group of genetic diseases caused by peroxisomal dysfunction, can be classified into three groups: (1) disorders of peroxisome biogenesis with a generalized loss of peroxisomal functions (Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum …
Peroxisomal disorders, a group of genetic diseases caused by peroxisomal dysfunction, can be classified into three groups: (1) disorders of …
Mouse models for peroxisome biogenesis defects and β-oxidation enzyme deficiencies.
Baes M, Van Veldhoven PP. Baes M, et al. Biochim Biophys Acta. 2012 Sep;1822(9):1489-500. doi: 10.1016/j.bbadis.2012.03.003. Epub 2012 Mar 14. Biochim Biophys Acta. 2012. PMID: 22446031 Free article. Review.
Because mice with generalized biogenesis defects seldom reach adulthood, conditional knockout models were generated to study the consequences of peroxisome deficiency in hepatocytes, different brain cell types and Sertoli cells. ...
Because mice with generalized biogenesis defects seldom reach adulthood, conditional knockout models were generated to study the consequence …
Peroxisome proliferator-activated receptors, coactivators, and downstream targets.
Qi C, Zhu Y, Reddy JK. Qi C, et al. Cell Biochem Biophys. 2000;32 Spring:187-204. doi: 10.1385/cbb:32:1-3:187. Cell Biochem Biophys. 2000. PMID: 11330046 Review.
A multifaceted approach, which includes the identification of additional coactivators that may be responsible for cell specific transcriptional activation of PPAR-mediated target genes, and generation of genetically modified animals (transgenic and gene disrupted), will be necess …
A multifaceted approach, which includes the identification of additional coactivators that may be responsible for cell specific transcriptio …
21 results