Modeling Disorders of Fatty Acid Metabolism in the Mouse (Q37850150)

24 August 2017

1 reference

title

Modeling disorders of fatty acid metabolism in the mouse (English)

1 reference

24 August 2017

Modeling Disorders of Fatty Acid Metabolism in the Mouse (English)

1 reference

https://api.crossref.org/works/10.1016/B978-0-12-384878-9.00010-8

26 November 2024

0 references

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A21377632

26 November 2024

Eric S. Goetzman

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A21377632

26 November 2024

language of work or name

English

1 reference

National Center for Biotechnology Information

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esummary.fcgi?db=pubmed&retmode=json&id=21377632

26 November 2024

publication date

1 January 2011

1 reference

24 August 2017

2011

1 reference

National Center for Biotechnology Information

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esummary.fcgi?db=pubmed&retmode=json&id=21377632

Progress in Molecular Biology and Translational Science

26 November 2024

published in

1 reference

24 August 2017

volume

100

1 reference

24 August 2017

page(s)

389-417

1 reference

Mitochondrial fatty acid oxidation disorders: pathophysiological studies in mouse models

24 August 2017

cites work

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26 November 2024

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Evidence for intermediate channeling in mitochondrial beta-oxidation

26 November 2024

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Isolation of a malonyl-CoA-sensitive CPT/beta-oxidation enzyme complex from heart mitochondria

26 November 2024

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26 November 2024

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26 November 2024

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Reaction of electron-transfer flavoprotein ubiquinone oxidoreductase with the mitochondrial respiratory chain

26 November 2024

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26 November 2024

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Evidence for Physical Association of Mitochondrial Fatty Acid Oxidation and Oxidative Phosphorylation Complexes

26 November 2024

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Short-chain acyl-coenzyme A dehydrogenase deficiency

26 November 2024

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Short-chain acyl-coenzyme A dehydrogenase deficiency in mice

26 November 2024

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Organic aciduria and butyryl CoA dehydrogenase deficiency in BALB/cByJ mice

26 November 2024

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Pathologic characterization of short-chain acyl-CoA dehydrogenase deficiency in BALB/cByJ mice

26 November 2024

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26 November 2024

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Abnormal nonshivering thermogenesis in mice with inherited defects of fatty acid oxidation

26 November 2024

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Targeted disruption of mouse long-chain acyl-CoA dehydrogenase gene reveals crucial roles for fatty acid oxidation

26 November 2024

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Long-chain acyl coenzyme A dehydrogenase deficiency: an inherited cause of nonketotic hypoglycemia

26 November 2024

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

26 November 2024

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Identification of very-long-chain acyl-CoA dehydrogenase deficiency in three patients previously diagnosed with long-chain acyl-CoA dehydrogenase deficiency

26 November 2024

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Mitochondrial long chain fatty acid beta-oxidation in man and mouse

26 November 2024

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Low expression of long-chain acyl-CoA dehydrogenase in human skeletal muscle

26 November 2024

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Long-chain acyl-CoA dehydrogenase is a key enzyme in the mitochondrial beta-oxidation of unsaturated fatty acids

26 November 2024

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Gestational, pathologic and biochemical differences between very long-chain acyl-CoA dehydrogenase deficiency and long-chain acyl-CoA dehydrogenase deficiency in the mouse

26 November 2024

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Differential induction of genes in liver and brown adipose tissue regulated by peroxisome proliferator-activated receptor-alpha during fasting and cold exposure in acyl-CoA dehydrogenase-deficient mice

26 November 2024

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Leptin: the "skinny" on torpor

26 November 2024

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Norepinephrine controls both torpor initiation and emergence via distinct mechanisms in the mouse

26 November 2024

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Influence of dietary fatty acid chain-length on metabolic tolerance in mouse models of inherited defects in mitochondrial fatty acid beta-oxidation

26 November 2024

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Mitochondrial dysfunction due to long-chain Acyl-CoA dehydrogenase deficiency causes hepatic steatosis and hepatic insulin resistance

26 November 2024

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Mitochondrial fatty acid oxidation disorders: clinical presentation of long-chain fatty acid oxidation defects before and after newborn screening

26 November 2024

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

26 November 2024

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26 November 2024

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Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress.

26 November 2024

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Optimal dietary therapy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency

26 November 2024

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Treatment recommendations in long-chain fatty acid oxidation defects: consensus from a workshop

26 November 2024

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Management and outcome in 75 individuals with long-chain fatty acid oxidation defects: results from a workshop

26 November 2024

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Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice

26 November 2024

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Carnitine supplementation induces long-chain acylcarnitine production--studies in the VLCAD-deficient mouse

26 November 2024

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Carnitine supplementation induces acylcarnitine production in tissues of very long-chain acyl-CoA dehydrogenase-deficient mice, without replenishing low free carnitine

26 November 2024

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Characterization of carnitine and fatty acid metabolism in the long-chain acyl-CoA dehydrogenase-deficient mouse

26 November 2024

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Medium-chain triglycerides impair lipid metabolism and induce hepatic steatosis in very long-chain acyl-CoA dehydrogenase (VLCAD)-deficient mice

26 November 2024

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Resistance to high-fat diet-induced obesity and insulin resistance in mice with very long-chain acyl-CoA dehydrogenase deficiency

26 November 2024

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

26 November 2024

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Peroxisome proliferator-activated receptor alpha mediates the adaptive response to fasting

26 November 2024

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Identification of novel peroxisome proliferator-activated receptor alpha (PPARalpha) target genes in mouse liver using cDNA microarray analysis

26 November 2024

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Neonatal screening for medium-chain acyl-CoA dehydrogenase deficiency

26 November 2024

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Newborn screening for MCAD deficiency: experience of the first three years in British Columbia, Canada.

26 November 2024

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26 November 2024

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Disturbed hepatic carbohydrate management during high metabolic demand in medium-chain acyl-CoA dehydrogenase (MCAD)-deficient mice

26 November 2024

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26 November 2024

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26 November 2024

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A missense mutation of mouse OCTN2, a sodium-dependent carnitine cotransporter, in the juvenile visceral steatosis mouse

26 November 2024

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26 November 2024

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26 November 2024

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Muscle Carnitine Palmityltransferase Deficiency and Myoglobinuria

26 November 2024

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Partial Deficiency of Muscle Carnitine Palmitoyltransferase with Normal Ketone Production

26 November 2024

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Homozygous carnitine palmitoyltransferase 1a (liver isoform) deficiency is lethal in the mouse

26 November 2024

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Homozygous carnitine palmitoyltransferase 1b (muscle isoform) deficiency is lethal in the mouse

26 November 2024

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Brain-specific carnitine palmitoyl-transferase-1c: role in CNS fatty acid metabolism, food intake, and body weight

26 November 2024

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Mitochondrial trifunctional protein deficiency: a severe fatty acid oxidation disorder with cardiac and neurologic involvement

26 November 2024

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Mitochondrial trifunctional protein defects: molecular basis and novel therapeutic approaches

26 November 2024

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Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death

26 November 2024

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Mice heterozygous for a defect in mitochondrial trifunctional protein develop hepatic steatosis and insulin resistance

26 November 2024

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Role of short-chain hydroxyacyl CoA dehydrogenases in SCHAD deficiency

26 November 2024

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Functional genomics of the beta-cell: short-chain 3-hydroxyacyl-coenzyme A dehydrogenase regulates insulin secretion independent of K+ currents.

26 November 2024

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3-Hydroxyacyl-coenzyme A dehydrogenase deficiency and hyperinsulinemic hypoglycemia: characterization of a novel mutation and severe dietary protein sensitivity.

26 November 2024

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2,4-Dienoyl-coenzyme A reductase deficiency: a possible new disorder of fatty acid oxidation

26 November 2024

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Disruption of mitochondrial beta -oxidation of unsaturated fatty acids in the 3,2-trans-enoyl-CoA isomerase-deficient mouse

26 November 2024

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Mitochondrial 2,4-dienoyl-CoA reductase deficiency in mice results in severe hypoglycemia with stress intolerance and unimpaired ketogenesis

26 November 2024

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Targeted disruption of the peroxisome proliferator-activated receptor alpha gene, PPAR alpha.

26 November 2024

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PPARalpha: energy combustion, hypolipidemia, inflammation and cancer

26 November 2024

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Conserved metabolic regulatory functions of sirtuins

26 November 2024

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Regulation of cellular metabolism by protein lysine acetylation

26 November 2024

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SIRT3 regulates mitochondrial fatty-acid oxidation by reversible enzyme deacetylation

26 November 2024

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Riboflavin and Mouse Hepatic Cell Structure and Function. Mitochondrial Oxidative Metabolism in Severe Deficiency States

26 November 2024

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Relationship between Hepatic Mitochondrial Oxidative Metabolism and Morphology during Riboflavin Deficiency and Recovery in Mice

26 November 2024

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26 November 2024

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

26 November 2024

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Chronic inhibition of fatty acid oxidation: new model of diastolic dysfunction

26 November 2024

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Gene expression profiling in livers of mice after acute inhibition of beta-oxidation.

26 November 2024

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Effects of carnitine palmitoyltransferase I inhibitors on hepatic hypertrophy

26 November 2024

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Animal models of steatosis

26 November 2024

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26 November 2024

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Antiketogenic and hypoglycemic effects of aminocarnitine and acylaminocarnitines

26 November 2024

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Carnitine effects on coenzyme A profiles in rat liver with hypoglycin inhibition of multiple dehydrogenases

26 November 2024

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Spiropentaneacetic acid as a specific inhibitor of medium-chain acyl-CoA dehydrogenase.

26 November 2024

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2-Mercaptoacetate administration depresses the beta-oxidation pathway through an inhibition of long-chain acyl-CoA dehydrogenase activity

26 November 2024

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Inhibition in vitro of acyl-CoA dehydrogenases by 2-mercaptoacetate in rat liver mitochondria

26 November 2024

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Mercaptoacetate inhibition of fatty acid beta-oxidation attenuates the oral acceptance of fat in BALB/c mice

26 November 2024

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Inhibition of fatty acid oxidation activates transforming growth factor-beta in cerebrospinal fluid and decreases spontaneous motor activity

26 November 2024

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Chemical knockout of pantothenate kinase reveals the metabolic and genetic program responsible for hepatic coenzyme A homeostasis

26 November 2024

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Abnormal fatty acid metabolism in patients in hopantenate therapy during clinical episodes

26 November 2024

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Systemic correction of a fatty acid oxidation defect by intramuscular injection of a recombinant adeno-associated virus vector

26 November 2024

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Recombinant adeno-associated virus-mediated gene delivery of long chain acyl coenzyme A dehydrogenase (LCAD) into LCAD-deficient mice

26 November 2024

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Biochemical correction of short-chain acyl-coenzyme A dehydrogenase deficiency after portal vein injection of rAAV8-SCAD.

26 November 2024

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Expression of short-chain acyl-CoA dehydrogenase (SCAD) proteins in the liver of SCAD deficient mice after hydrodynamic gene transfer

26 November 2024

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Synergistic heterozygosity: disease resulting from multiple partial defects in one or more metabolic pathways

26 November 2024

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Synergistic heterozygosity in mice with inherited enzyme deficiencies of mitochondrial fatty acid beta-oxidation

26 November 2024

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Fatty acid metabolism pathway play an important role in carcinogenesis of human colorectal cancers by Microarray-Bioinformatics analysis

26 November 2024

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The role of enoyl-CoA hydratase short chain 1 and peroxiredoxin 3 in PP2-induced apoptosis in human breast cancer MCF-7 cells

26 November 2024

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In vitro studies on the inhibition of colon cancer by butyrate and carnitine.

26 November 2024

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Acyl-coenzyme A dehydrogenases are localized on GLUT4-containing vesicles via association with insulin-regulated aminopeptidase in a manner dependent on its dileucine motif

26 November 2024

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Identifiers

1 reference

24 August 2017

1 reference