(Q37130100)

18 August 2017

1 reference

title

PPARs as therapeutic targets for correction of inborn mitochondrial fatty acid oxidation disorders (English)

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18 August 2017

0 references

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F Djouadi

1

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18 August 2017

J Bastin

series ordinal

2

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18 August 2017

publication date

4 April 2008

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Journal of Inherited Metabolic Disease

18 August 2017

published in

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18 August 2017

volume

31

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18 August 2017

issue

2

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18 August 2017

page(s)

217-225

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Targeted disruption of the alpha isoform of the peroxisome proliferator-activated receptor gene in mice results in abolishment of the pleiotropic effects of peroxisome proliferators

18 August 2017

cites work

1 reference

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Bezafibrate is a dual ligand for PPARalpha and PPARbeta: studies using null mice

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10545-008-0844-7

Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride

21 January 2018

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https://api.crossref.org/works/10.1007%2FS10545-008-0844-7

Management of fatty acid oxidation disorders: a survey of current treatment strategies

21 January 2018

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12 December 2020

Diagnosis and management of defects of mitochondrial beta-oxidation

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12 December 2020

Steady-state kinetics of bezafibrate and clofibrate in healthy female volunteers

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12 December 2020

Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects

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12 December 2020

Peroxisome proliferator-activated receptor alpha target genes

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Bezafibrate. A review of its pharmacodynamic and pharmacokinetic properties, and therapeutic use in hyperlipidaemia

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12 December 2020

Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency.

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Screening newborns for inborn errors of metabolism by tandem mass spectrometry

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Activation of a member of the steroid hormone receptor superfamily by peroxisome proliferators

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Comparative pharmacokinetics of two oral bezafibrate preparations

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Switching from insulin to oral sulfonylureas in patients with diabetes due to Kir6.2 mutations.

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12 December 2020

Correlation between genotype, metabolic data, and clinical presentation in carnitine palmitoyltransferase 2 (CPT2) deficiency.

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12 December 2020

Fetal fatty acid oxidation disorders, their effect on maternal health and neonatal outcome: impact of expanded newborn screening on their diagnosis and management

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12 December 2020

Carnitine palmitoyltransferase deficiencies

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12 December 2020

Bezafibrate in men with lower extremity arterial disease: randomised controlled trial

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12 December 2020

Regulation of muscle fiber type and running endurance by PPARdelta

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12 December 2020

Dual and pan-peroxisome proliferator-activated receptors (PPAR) co-agonism: the bezafibrate lessons

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12 December 2020

Clear relationship between ETF/ETFDH genotype and phenotype in patients with multiple acyl-CoA dehydrogenation deficiency

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12 December 2020

Genetic basis for correction of very-long-chain acyl-coenzyme A dehydrogenase deficiency by bezafibrate in patient fibroblasts: toward a genotype-based therapy

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12 December 2020

Correction of fatty acid oxidation in carnitine palmitoyl transferase 2-deficient cultured skin fibroblasts by bezafibrate

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12 December 2020

Muscle carnitine palmitoyltransferase II deficiency: clinical and molecular genetic features and diagnostic aspects

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Pharmacological enhancement of mutated alpha-glucosidase activity in fibroblasts from patients with Pompe disease

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12 December 2020

PPAR delta: a dagger in the heart of the metabolic syndrome

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12 December 2020

Decrease in triglyceride level by bezafibrate is related to reduction of recurrent coronary events: a Bezafibrate Infarction Prevention substudy

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12 December 2020

Identification of 16 new disease-causing mutations in the CPT2 gene resulting in carnitine palmitoyltransferase II deficiency

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12 December 2020

From molecular action to physiological outputs: peroxisome proliferator-activated receptors are nuclear receptors at the crossroads of key cellular functions

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12 December 2020

Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders.

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12 December 2020

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

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12 December 2020

Strategies for the diagnosis of mitochondrial fatty acid beta-oxidation disorders

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12 December 2020

Task force guidelines handbook: EFNS guidelines on diagnosis and management of fatty acid mitochondrial disorders

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12 December 2020

The peroxisome proliferator-activated receptor regulates mitochondrial fatty acid oxidative enzyme gene expression

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12 December 2020

Defects of mitochondrial beta-oxidation: a growing group of disorders.

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12 December 2020

Fatty acid oxidation disorders

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12 December 2020

Mutation analysis in mitochondrial fatty acid oxidation defects: Exemplified by acyl-CoA dehydrogenase deficiencies, with special focus on genotype-phenotype relationship

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12 December 2020

Recent advances in newborn screening

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12 December 2020

Recognition and management of fatty acid oxidation defects: a series of 107 patients.

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12 December 2020

Fatty acid oxidation defects as a cause of neuromyopathic disease in infants and adults

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12 December 2020

Minireview: lipid metabolism, metabolic diseases, and peroxisome proliferator-activated receptors

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12 December 2020

Differential sensitivity of the cystic fibrosis (CF)-associated mutants G551D and G1349D to potentiators of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel

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12 December 2020

Protein-misfolding diseases and chaperone-based therapeutic approaches

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12 December 2020

The mechanisms of action of PPARs

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12 December 2020

Peroxisome proliferator-activated receptor delta controls muscle development and oxidative capability

1 reference

12 December 2020

Peroxisome proliferator activated receptor delta (PPARdelta) agonist but not PPARalpha corrects carnitine palmitoyl transferase 2 deficiency in human muscle cells

1 reference

12 December 2020

1 reference

12 December 2020

Bezafibrate induces FALDH in human fibroblasts; implications for Sjögren-Larsson syndrome

1 reference

12 December 2020

10.1007/S10545-008-0844-7

Identifiers

DOI

1 reference

release_oqqd3wzgkrg4jf3myur2rdqz3e

18 August 2017

Fatcat ID

1 reference

Fatcat

https://api.fatcat.wiki/v0/release/oqqd3wzgkrg4jf3myur2rdqz3e

24 November 2022

mapped directly with Wikidata item

1 reference