Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

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

Pediatric research - 1 Jun 2005

Spiekerkoetter Ute, Tokunaga Chonan, Wendel Udo, Mayatepek Ertan, Ijlst Lodewijk, Vaz Frederic M, van Vlies Naomi, Overmars Henk, Duran Marinus, Wijburg Frits A, Wanders Ronald J, Strauss Arnold W

Abstract excerpt

Deficiency of very-long-chain acyl-CoA dehydrogenase (VLCAD) is the most common long-chain fatty acid oxidation defect and presents with heterogeneous clinical manifestations. Accumulation of long-chain acylcarnitines and deficiency of free carnitine have often been proposed to play an important role in disease pathogenesis. The VLCAD-deficient mouse exhibits similar clinical and biochemical phenotypes to those...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Acyl-CoA Dehydrogenase, Long-Chain
Share to Bile

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Carnitine

Share to Homeostasis

Share to Lipid Metabolism, Inborn Errors

Share to Mice, Knockout

Share to Muscle, Skeletal

Source

Identifiers

PMID: 15774826
DOI: 10.1203/01.PDR.0000157915.26049.47

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice.PMID 15774826Read ↗

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

Slc22a5 haploinsufficiency does not aggravate the phenotype of the long-chain acyl-CoA dehydrogenase KO mouse.Semantic proximity Read ↗

Tissue-specific strategies of the very-long chain acyl-CoA dehydrogenase-deficient (VLCAD-/-) mouse to compensate a defective fatty acid β-oxidation.Semantic proximity Read ↗

Acute arrhythmias in a long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency mouse model.Semantic proximity Read ↗

Long-term correction of very long-chain acyl-coA dehydrogenase deficiency in mice using AAV9 gene therapy.Semantic proximity Read ↗

Global Analysis of Plasma Lipids Identifies Liver-Derived Acylcarnitines as a Fuel Source for Brown Fat Thermogenesis.Semantic proximity Read ↗

Systematic assessment of lipid profiles for the discovery of tissue contributors to the circulating lipid pool in cold exposureSemantic proximity Read ↗

Very-long-chain acyl-coenzyme a dehydrogenase deficiency in mice.Semantic proximity Read ↗

Role of choline deficiency in the Fatty liver phenotype of mice fed a low protein, very low carbohydrate ketogenic diet.Semantic proximity Read ↗

Mitochondrial fatty acid oxidation disorders: pathophysiological studies in mouse models.Semantic proximity Read ↗

Food withdrawal lowers energy expenditure and induces inactivity in long-chain fatty acid oxidation-deficient mouse models.Semantic proximity Read ↗

Diagnostic assessment and long-term follow-up of 13 patients with Very Long-Chain Acyl-Coenzyme A dehydrogenase (VLCAD) deficiency.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.