Fatty Acid Accumulation and Resulting PPARα Activation in Fibroblasts due to Trifunctional Protein Deficiency (Q42175960)

18 October 2017

Fatty Acid Accumulation and Resulting PPARα Activation in Fibroblasts due to Trifunctional Protein Deficiency (English)

1 reference

Trifunctional protein deficiency

18 October 2017

0 references

author name string

Masato Wakabayashi

1

1 reference

18 October 2017

Yuji Kamijo

2

1 reference

18 October 2017

Takero Nakajima

3

1 reference

18 October 2017

Naoki Tanaka

4

1 reference

18 October 2017

Eiko Sugiyama

5

1 reference

18 October 2017

Tian Yangyang

6

1 reference

18 October 2017

Takefumi Kimura

7

1 reference

18 October 2017

Toshifumi Aoyama

8

1 reference

18 October 2017

8 May 2012

1 reference

18 October 2017

1 reference

18 October 2017

2012

1 reference

18 October 2017

371691

1 reference

Creative Commons Attribution 3.0 Unported

18 October 2017

start time

1 January 2012

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

1 reference

April 2022 Public Data File from Crossref

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Inhibition of peroxisome-proliferator-activated receptor (PPAR)alpha by MK886

26 October 2017

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Fatty acid regulation of gene transcription

26 October 2017

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Regulation of lipid and lipoprotein metabolism by PPAR activators

26 October 2017

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Molecular recognition of fatty acids by peroxisome proliferator-activated receptors

26 October 2017

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Altered constitutive expression of fatty acid-metabolizing enzymes in mice lacking the peroxisome proliferator-activated receptor alpha (PPARalpha)

26 October 2017

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Genomic and mutational analysis of the mitochondrial trifunctional protein beta-subunit (HADHB) gene in patients with trifunctional protein deficiency

26 October 2017

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Fatty acids and eicosanoids regulate gene expression through direct interactions with peroxisome proliferator-activated receptors alpha and gamma

26 October 2017

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Hypolipidemic drugs, polyunsaturated fatty acids, and eicosanoids are ligands for peroxisome proliferator-activated receptors alpha and delta

26 October 2017

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Molecular characterization of mitochondrial trifunctional protein deficiency: formation of the enzyme complex is important for stabilization of both alpha- and beta-subunits

26 October 2017

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Differential expression of peroxisome proliferator-activated receptors (PPARs): tissue distribution of PPAR-alpha, -beta, and -gamma in the adult rat

26 October 2017

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A novel disease with deficiency of mitochondrial very-long-chain acyl-CoA dehydrogenase

26 October 2017

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Rat very-long-chain acyl-CoA dehydrogenase, a novel mitochondrial acyl-CoA dehydrogenase gene product, is a rate-limiting enzyme in long-chain fatty acid beta-oxidation system. cDNA and deduced amino acid sequence and distinct specificities of [...]

26 October 2017

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Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients

26 October 2017

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Cloning of human very-long-chain acyl-coenzyme A dehydrogenase and molecular characterization of its deficiency in two patients

26 October 2017

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Genetic deficiency of short-chain acyl-coenzyme A dehydrogenase in cultured fibroblasts from a patient with muscle carnitine deficiency and severe skeletal muscle weakness

26 October 2017

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Medium-chain acyl-CoA dehydrogenase deficiency. Diagnosis by stable-isotope dilution measurement of urinary n-hexanoylglycine and 3-phenylpropionylglycine

26 October 2017

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Short chain acyl-coenzyme A dehydrogenase (SCAD) deficiency. Immunochemical demonstration of molecular heterogeneity due to variant SCAD with differing stability

26 October 2017

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Cytochrome P-450 hPCN3, a novel cytochrome P-450 IIIA gene product that is differentially expressed in adult human liver. cDNA and deduced amino acid sequence and distinct specificities of cDNA-expressed hPCN1 and hPCN3 for the metabolism of [...]

26 October 2017

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Combined enzyme defect of mitochondrial fatty acid oxidation

26 October 2017

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Pretreatment by low-dose fibrates protects against acute free fatty acid-induced renal tubule toxicity by counteracting PPARα deterioration.

26 October 2017

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Lipid extraction of tissues with a low-toxicity solvent.

29 May 2018

1 reference

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Peroxisome proliferator-activated receptor alpha target genes.

29 May 2018

1 reference

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Hepatitis C virus core protein induces spontaneous and persistent activation of peroxisome proliferator-activated receptor alpha in transgenic mice: implications for HCV-associated hepatocarcinogenesis

17 August 2018

1 reference

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Identification of functions of peroxisome proliferator-activated receptor alpha in proximal tubules.

17 August 2018

1 reference

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Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the alpha-subunit of the mitochondrial trifunctional protein

17 August 2018

2 references

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17 August 2018

12 December 2020

Human trifunctional protein deficiency: A new disorder of mitochondrial fatty acid β-oridation

1 reference

Crossref

https://api.crossref.org/works/10.1155%2F2012%2F371691

Immunochemical characterization of variant medium-chain acyl-CoA dehydrogenase in fibroblasts from patients with medium-chain acyl-CoA dehydrogenase deficiency

21 January 2018

1 reference

12 December 2020

PPARalpha protects proximal tubular cells from acute fatty acid toxicity

1 reference

12 December 2020

An acyl-coenzyme A dehydrogenase assay utilizing the ferricenium ion

1 reference

12 December 2020

Purification and Properties of Rat Liver Acyl-CoA Dehydrogenases and Electron Transfer Flavoprotein1

1 reference

12 December 2020

Identifiers

DOI

10.1155/2012/371691

1 reference

18 October 2017

1 reference

18 October 2017

1 reference