Secondary carnitine deficiency and impaired docosahexaenoic (22:6n-3) acid synthesis: a common denominator in the pathophysiology of diseases of oxidative phosphorylation and beta-oxidation (Q33843176)

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English	Secondary carnitine deficiency and impaired docosahexaenoic (22:6n-3) acid synthesis: a common denominator in the pathophysiology of diseases of oxidative phosphorylation and beta-oxidation	scientific article

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scholarly article

1 reference

Europe PubMed Central

29 July 2017

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Europe PubMed Central

Secondary carnitine deficiency and impaired docosahexaenoic (22:6n-3) acid synthesis: a common denominator in the pathophysiology of diseases of oxidative phosphorylation and beta-oxidation (English)

1 reference

Europe PubMed Central

29 July 2017

pathophysiology

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based on heuristic

inferred from title

phosphorylation

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based on heuristic

inferred from title

author name string

Infante JP

1

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Europe PubMed Central

29 July 2017

Huszagh VA

2

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Europe PubMed Central

29 July 2017

language of work or name

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publication date

1 February 2000

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Europe PubMed Central

29 July 2017

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Europe PubMed Central

29 July 2017

468

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Europe PubMed Central

29 July 2017

1

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Europe PubMed Central

29 July 2017

1-5

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Europe PubMed Central

29 July 2017

Mitochondrial diseases in man and mouse

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Neonatal presentations of mitochondrial metabolic disorders

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Docosahexaenoate-containing phospholipids in sarcoplasmic reticulum and retinal photoreceptors. A proposal for a role in Ca2+-ATPase calcium transport

1 reference

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On the molecular etiology of decreased arachidonic (20:4n-6), docosapentaenoic (22:5n-6) and docosahexaenoic (22:6n-3) acids in Zellweger syndrome and other peroxisomal disorders

1 reference

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Oxidative phosphorylation at the fin de siècle

1 reference

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A function for the vitamin E metabolite alpha-tocopherol quinone as an essential enzyme cofactor for the mitochondrial fatty acid desaturases

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Primary and secondary alterations of neonatal carnitine metabolism.

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Analysis of the putative role of 24-carbon polyunsaturated fatty acids in the biosynthesis of docosapentaenoic (22:5n-6) and docosahexaenoic (22:6n-3) acids

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Pyruvate dehydrogenase, substrate specificity and product inhibition.

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The effect of respiratory chain impairment of beta-oxidation in rat heart mitochondria

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Impaired mitochondrial beta-oxidation in a patient with an abnormality of the respiratory chain. Studies in skeletal muscle mitochondria

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Fatty acid oxidation in fibroblasts from patients with defects in beta-oxidation and in the respiratory chain.

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Influence of docosahexaenoic acid on cerebral lipid peroxide level in aged rats with and without hypercholesterolemia.

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Clinical and biochemical aspects of carnitine deficiency and insufficiency: transport defects and inborn errors of beta-oxidation

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Disorders of mitochondrial long-chain fatty acid oxidation

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Retinal dystrophy in long chain 3-hydroxy-acyl-coA dehydrogenase deficiency

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Ophthalmologic findings in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency caused by the G1528C mutation: a new type of hereditary metabolic chorioretinopathy.

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Dietary management of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD). A case report and survey

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Docosahexaenoic acid and retinal function in children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.

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Polyunsaturated fatty acid lipidosis infantile form of so-called neuronal ceroidlipofuscinosis.

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Polyunsaturated fatty acid lipidosis. II. Lipid biochemical studies

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Systemic carnitine deficiency exacerbated by a strict vegetarian diet

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Risks of alternative nutrition in infancy: a case report of severe iodine and carnitine deficiency

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Dietary manipulation of long-chain polyunsaturated fatty acids in the retina and brain of guinea pigs

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Dietary Reference Intakes: the new basis for recommendations for calcium and related nutrients, B vitamins, and choline

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Biosynthesis of acyl-specific glycerophospholipids in mammalian tissues. Postulation of new pathways

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Docosahexaenoic acid therapy in docosahexaenoic acid-deficient patients with disorders of peroxisomal biogenesis.

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21 January 2018

MRI evidence that docosahexaenoic acid ethyl ester improves myelination in generalized peroxisomal disorders.

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Therapy of mitochondrial disorders

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Brain docosahexaenoate accretion in fetal baboons: bioequivalence of dietary alpha-linolenic and docosahexaenoic acids.

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21 January 2018

Fatty acid composition of brain glycerophospholipids in peroxisomal disorders

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

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A murine model for juvenile NCL: gene targeting of mouse Cln3

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Chronic dietary choline modulates synaptic plasticity in the cerebellar glomeruli of aging mice.

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Direct measurement of high-energy phosphate compounds in patients with neuromuscular disease

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The relationship between alpha-tocopherol and phospholipid fatty acids in rat liver subcellular membrane fractions

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Hypothyroidism and thyroxin substitution affect the n−3 fatty acid composition of rat liver mitochondria

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Tocopherol in inborn errors of intermediary metabolism

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A mouse model for mitochondrial myopathy and cardiomyopathy resulting from a deficiency in the heart/muscle isoform of the adenine nucleotide translocator

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Mitochondrial myopathy with lactic acidosis and deficient activity of muscle succinate cytochrome-c-oxidoreductase

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Muscle carnitine deficiency and lipid storage myopathy in patients with mitochondrial myopathy

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Ultrastructural and histochemical abnormalities of skeletal muscle in patients with chronic vitamin E deficiency

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Effects of a prolonged vitamin E deficiency in the rat.

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Vitamin E in a mitochondrial myopathy with proliferating mitochondria

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Fatty acid oxidation defects in muscle

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Pathology of skeletal muscle and impaired respiratory chain function in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency with the G1528C mutation

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Long-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency neuropathy: response to cod liver oil

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Inhibitory effect of 3-hydroxyacyl-CoAs and other long-chain fatty acidβ-oxidation intermediates on mitochondrial oxidative phosphorylation

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Superoxide dismutase, catalase and glutathione peroxidase in infantile, late infantile and juvenile neuronal ceroid-lipofuscinosis

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Decreased plasma carnitine and trimethyl-L-lysine levels associated with lysosomal accumulation of a trimethyl-L-lysine containing protein in Batten disease

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Dietary docosahexaenoic acid has little effect on peroxisomes in healthy mice

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10.1016/S0014-5793(00)01083-8

1 reference

Europe PubMed Central

29 July 2017

1 reference

Europe PubMed Central

29 July 2017

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