Acyl coa dehydrogenase long chain. Medical search. Frequent questions

Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is a condition that prevents the body from converting certain fats into energy, particularly during periods without food (fasting). (medlineplus.gov)
Very-Long-Chain Acyl-CoA dehydrogenase (VLCAD) deficiency is an inherited metabolic disorder caused by mutations in the ACADVL gene. (myriad.com)
All three types of VLCAD deficiency are caused by an error in the production of an enzyme called very-long-chain acyl-coenzyme A dehydrogenase. (myriad.com)
What Is the Prognosis for an Individual with Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency? (myriad.com)
Schiff M, Mohsen AW, Karunanidhi A, McCracken E, Yeasted R, Vockley J. Molecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiency. (medscape.com)
Scalais E, Bottu J, Wanders RJ, Ferdinandusse S, Waterham HR, De Meirleir L. Familial very long chain acyl-CoA dehydrogenase deficiency as a cause of neonatal sudden infant death: Improved survival by prompt diagnosis. (medscape.com)
Long-chain 3-hydroxyacyl CoA dehydrogenase deficiency and choroidal neovascularization]. (medscape.com)
Clinical, biochemical, and morphologic investigations of a case of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. (medscape.com)
Peripheral sensory-motor polyneuropathy, pigmentary retinopathy, and fatal cardiomyopathy in long-chain 3-hydroxy-acyl-CoA dehydrogenase deficiency. (medscape.com)
Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: clinical presentation and follow-up of 50 patients. (medscape.com)
Acute dilated cardiomyopathy in a patient with deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase. (medscape.com)
Optimal dietary therapy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. (medscape.com)
Gillingham MB, Purnell JQ, Jordan J, Stadler D, Haqq AM, Harding CO. Effects of higher dietary protein intake on energy balance and metabolic control in children with long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiency. (medscape.com)
Gillingham MB, Scott B, Elliott D, Harding CO. Metabolic control during exercise with and without medium-chain triglycerides (MCT) in children with long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiency. (medscape.com)
IJlst L, Wanders RJ, Ushikubo S, Kamijo T, Hashimoto T. 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. (medscape.com)
Pigmentary retinopathy in long chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. (medscape.com)
Infants suspected to have very-long chain acyl-CoA dehydrogenase deficiency from newborn screening. (medscape.com)
MCT oil-based diet reverses hypertrophic cardiomyopathy in a patient with very long chain acyl-coA dehydrogenase deficiency. (medscape.com)
In 1983, Gregersen et al demonstrated a medium-chain acyl-coenzyme A (CoA) dehydrogenase (MCAD) deficiency in a patient with hypoketotic hypoglycemia. (medscape.com)
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency Fatty acid oxidation disorders are lipid metabolism disorders that are caused by a lack or deficiency of the enzymes needed to break down fats, resulting in delayed mental and physical development. (msdmanuals.com)
It has also been linked to an inherited enzyme deficiency called long chain acyl-CoA dehydrogenase (LCHAD) in the baby. (britishlivertrust.org.uk)
We were concerned in two areas at that time, one being the long term effect of frequent breakdown of muscle tissue in Adam's body, and the other the long-term effect of a deficiency of DHA (4) on his retinas. (fodsupport.org)
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency, Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency), amino acid disorders (e.g. (luriechildrens.org)
In humans, acetoacetyl-CoA is involved in the metabolic disorder called the short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (HADH) pathway. (hmdb.ca)
Fatty acid oxidation disorders identified included very long chain acyl-CoA dehydrogenase deficiency, medium chain acyl-CoA dehydrogenase deficiency and carnitine transporter defects. (bmj.com)
Short-chain acyl-CoA dehydrogenase deficiency (SCAD) is a condition in which the body is unable to break down certain fats. (babysfirsttest.org)
Short-chain acyl-CoA dehydrogenase deficiency (SCAD) is estimated to affect one in 40,000 to 100,000 newborns. (babysfirsttest.org)
If your baby's newborn screening result for short-chain acyl-CoA dehydrogenase deficiency (SCAD) was out of the normal range, your baby's doctor or the state screening program will contact you to arrange for your child to have additional testing. (babysfirsttest.org)
Each child with short-chain acyl-CoA dehydrogenase deficiency (SCAD) has a different experience. (babysfirsttest.org)
Some children with short chain acyl-CoA dehydrogenase deficiency (SCAD) take prescription L-carnitine supplements. (babysfirsttest.org)
Children who are treated early for short-chain acyl-CoA dehydrogenase deficiency (SCAD) can have healthy growth and development. (babysfirsttest.org)
Medium-chain acyl-CoA dehydrogenase deficiency (MCAD) is an autosomal recessively inherited disorder of ß-oxidation of fatty acids. (medicover-genetics.com)
MCAD deficiency is caused by pathogenic variants in the medium-chain acyl-CoA dehydrogenase gene ( ACADM ). (medicover-genetics.com)
Despite advances in newborn screening and treatment of fatty acid oxidation disorders, patients with very-long-chain acyl-CoA dehydrogenase deficiency (VLCADD) continue to suffer from heart failure. (mitoaction.org)
Development of a newborn screening follow-up algorithm for the diagnosis of isobutyryl-CoA dehydrogenase deficiency. (harvard.edu)
Coordinated and reversible reduction of enzymes involved in terminal oxidative metabolism in skeletal muscle mitochondria from a riboflavin-responsive, multiple acyl-CoA dehydrogenase deficiency patient. (harvard.edu)
Anesthetic considerations for a patient with compound heterozygous medium-chain Acyl-CoA dehydrogenase deficiency. (harvard.edu)
Compound heterozygosity in four asymptomatic siblings with medium-chain acyl-CoA dehydrogenase deficiency. (harvard.edu)
Short-chain acyl-CoA dehydrogenase deficiency: a cause of ophthalmoplegia and multicore myopathy. (harvard.edu)
[ 1 ] Intracellular carnitine deficiency impairs the entry of long-chain fatty acids into the mitochondrial matrix. (medscape.com)
The five most commonly diagnosed conditions by newborn screen in the United States are 1) hearing loss, 2) primary congenital hypothyroidism, 3) cystic fibrosis, 4) sickle cell disease, and 5) medium-chain acyl-CoA dehydrogenase (MCAD) deficiency 3 . (acog.org)
Clinical and biochemical outcome of patients with very long-chain acyl-CoA dehydrogenase deficiency. (cdc.gov)

This acyl-CoA is linked to carnitine by the action of CPT I, with simultaneous transport across the mitochondrial membrane barrier. (medscape.com)
How do Fatty Acyl-CoA's pass the mitochondrial intermembrane space? (flashcardmachine.com)
In early type-2 diabetic hearts, flux through the fatty acid oxidation pathway was increased as a result of increased cellular uptake (CD36), mitochondrial uptake (CPT1B), as well as increased β-hydroxyacyl-CoA dehydrogenase and medium-chain acyl-CoA dehydrogenase activities, despite reduced mitochondrial mass. (oroboros.at)
To date, there have been no randomized controlled trials that have examined the effects of long-term CR on muscle mitochondrial function in humans. (medscape.com)

Once inside the mitochondrion, the action of CPT II at the inner surface of the membrane releases free carnitine, which exits to the cytosol and leaves behind the acyl-CoA molecule. (medscape.com)
Carnitine binds acyl residues and helps in their elimination, decreasing the number of acyl residues conjugated with coenzyme A (CoA) and increasing the ratio between free and acylated CoA. (medscape.com)

enoyl-CoA hydratase 1 [Source:HGNC Symb. (gsea-msigdb.org)

At the end of our last story we were starting to be concerned about long-term effects of LCHAD. (fodsupport.org)

Long-chain acyl-CoA dehydrogenase (EC 1.3.8.8, palmitoyl-CoA dehydrogenase, palmitoyl-coenzyme A dehydrogenase, long-chain acyl-coenzyme A dehydrogenase, long-chain-acyl-CoA:(acceptor) 2,3-oxidoreductase, ACADL (gene). (wikipedia.org)
On the mechanism of dehydrogenation of fatty acyl derivatives of coenzyme A. III. (wikipedia.org)
Hauge, J.G., Crane, F.L. and Beinert, H. On the mechanism of dehydrogenation of fatty acyl derivatives of coenzyme A. III. (enzyme-database.org)
It is a substrate for succinyl-CoA:3-ketoacid-coenzyme A transferase, hydroxymethylglutaryl-CoA synthase, short-chain 3-hydroxyacyl-CoA dehydrogenase, peroxisomal bifunctional enzyme, acetyl-CoA acetyltransferase, and 3-ketoacyl-CoA thiolase. (hmdb.ca)
Long-chain acyl coenzyme A dehydrogenase (ACADL) is the first step in catalytic fatty acid oxidation and plays an important role in long-chain fatty acid oxidation including expression regulation and activity regulation. (animbiosci.org)

Hydroxyacyl-CoA Dehydrogenase (HADHA) and Long Chain Acyl-CoA Dehydrogenase (LCAD) catalyze the oxidation of long chain fatty acids, whereas pyruvate dehydrogenase (PDH) catalyzes glucose oxidation in the heart. (wvu.edu)

is an enzyme with systematic name long-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase. (wikipedia.org)
This enzyme catalyses the following chemical reaction a long-chain acyl-CoA + electron-transfer flavoprotein ⇌ {\displaystyle \rightleftharpoons } a long-chain trans-2,3-dehydroacyl-CoA + reduced electron-transfer flavoprotein This enzyme contains FAD as prosthetic group and participates in fatty acid metabolism and PPAR signaling pathway. (wikipedia.org)
This gene provides instructions for making an enzyme called very long-chain acyl-CoA dehydrogenase, which is required to break down (metabolize) a group of fats called very long-chain fatty acids. (medlineplus.gov)
When cells do not have enough of this enzyme, very long-chain fatty acids are not broken down properly. (medlineplus.gov)
This enzyme is responsible for breaking down a type of fat known as very-long-chain fatty acids and converting it into energy. (myriad.com)
Entry into the beta-oxidation cycle requires the action of acyl-CoA dehydrogenase, the first enzyme in the sequence, which removes electrons from the alpha-carbon and the beta-carbon, introducing a double bond. (medscape.com)
The enzyme from pig liver can accept substrates with acyl chain lengths of 6 to at least 16 carbon atoms. (enzyme-database.org)

Purification and characterization of short-chain, medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. (wikipedia.org)
It plays an essential role in the transfer of long-chain fatty acids into the mitochondria for beta-oxidation. (medscape.com)
Regulation of the intramitochondrial free CoA also is affected, with accumulation of acyl-CoA esters in the mitochondria. (medscape.com)

In the cytosol, a saturated, straight-chain fatty acid molecule with no double bonds is activated by the action of fatty acyl-CoA synthetase to form its corresponding acyl-CoA. (medscape.com)
Acyl CoA Synthetase: What is it/What does it do? (flashcardmachine.com)
acyl-CoA synthetase short chain family m. (gsea-msigdb.org)

Cleavage of the 3-keto compound at the now unstable alpha-beta carbon bond and transfer of another CoA moiety to the new fragment results in 2 products: acetyl-CoA, composed of the carbonyl and original alpha-carbon from the starting molecule, and a new fatty acyl-CoA that is 2 carbons shorter than the original molecule. (medscape.com)
Acetyl CoA is generated from fatty acids through repeated beta-oxidation cycles. (msdmanuals.com)

The aim of this study was to clone the mRNA sequence of the Acyl-CoA dehydrogenase long chain ( ACADL ) gene of goats and explore the effect of ACADL on the differentiation of subcutaneous fat cells on this basis. (animbiosci.org)
We obtained the ACADL gene of goats by cloning and used quantitative real-time polymerase chain reaction (qPCR) to detect the ACADL expression patterns of different goat tissues and subcutaneous fat cells at different lipid induction stages. (animbiosci.org)

Consequently, long-chain fatty acids are not available for beta-oxidation and energy production, and the production of ketone bodies (which are used by the brain) is also impaired. (medscape.com)

a long-chain acyl-CoA = an acyl-CoA thioester where the acyl chain contains 13 to 22 carbon atoms. (enzyme-database.org)

Sets of 4 enzymes (an acyl dehydrogenase, a hydratase, a hydroxyacyl dehydrogenase, and a lyase) specific for different chain lengths (very long chain, long chain, medium chain, and short chain) are required to catabolize fatty acids completely. (msdmanuals.com)

Very long-chain fatty acids or partially metabolized fatty acids may also build up in tissues and damage the heart, liver, and muscles. (medlineplus.gov)
In addition, a buildup of very-long-chain fatty acids in the body can damage the heart, liver, and muscles, causing additional symptoms of the disease. (myriad.com)
Over the long term, children have delayed mental and physical development, an enlarged liver, heart muscle weakness, and an irregular heartbeat. (msdmanuals.com)

Management and outcome in 75 individuals with long-chain fatty acid oxidation defects: results from a workshop. (medscape.com)
Measurement of tissue acyl-CoAs using flow-injection tandem mass spectrometry: acyl-CoA profiles in short-chain fatty acid oxidation defects. (harvard.edu)

This indicates that the heart is no longer capable of further increasing its capacity for fatty acid oxidation. (oroboros.at)

With attention focused on the definition of additional disorders, researchers described patients with a Reye syndrome-like presentation who excreted dicarboxylic acids of chain lengths C6-C10 in their urine. (medscape.com)
In 2015 new evidence was assessed and it was agreed by clinical experts and the NSU that a group of rare disorders (carboxylase deficiencies) should no longer be screened for. (nsu.govt.nz)
The goal of this public health program is to decrease morbidity and mortality by screening for disorders in which early intervention will improve neonatal and long-term health outcomes. (acog.org)

In addition, quantitative real-time polymerase chain reaction (qPCR) was used to detect the expression patterns of ACADL in various tissues and subcutaneous adipocytes at different stages of differentiation. (animbiosci.org)

Acyl-CoA Dehydrogenases" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (harvard.edu)

Long-chain acylcarnitines are also toxic and may have an arrhythmogenic effect, causing sudden cardiac death. (medscape.com)

EC 1.3.8.1 , short-chain acyl-CoA dehydrogenase, EC 1.3.8.8, medium-chain acyl-CoA dehydrogenase, and EC 1.3.8.9 , very-long-chain acyl-CoA dehydrogenase. (enzyme-database.org)

Long-chain acyl-CoA dehydrogenase activity was slightly decreased, resulting in the accumulation of long-chain acylcarnitine species. (oroboros.at)

In the process, another electron transfer occurs, this time to nicotinamide-adenine dinucleotide (NAD), and more ATP is produced by passage down the electron transport chain. (medscape.com)

The lack of these enzymes leaves the body short of energy and allows breakdown products, such as acyl-CoA, to accumulate. (msdmanuals.com)

Acetoacetyl-CoA is an intermediate in the metabolism of butanoate. (hmdb.ca)

Oxidation of the hydroxyl substituent group on the beta-carbon creates an inherently unstable beta-ketoacyl-CoA compound. (medscape.com)

Season-dependent differentially phosphorylated proteins were identified via MALDI-TOF/TOF MS (F1-ATPase α-chain, long-chain specific acyl-CoA dehydrogenase, ornithine transcarbamylase) and potential hibernation-specific roles are discussed. (uwo.ca)

Acetoacetyl-CoA is a strong basic compound (based on its pKa). (hmdb.ca)

In a subset of individuals, a muscle biopsy was performed for transcriptional profiling via quantitative reverse transcription polymerase chain reaction and microarrays. (medscape.com)

After a certain amount of activity though these short term energy sources become depleted and unless more carbohydrate sources are digested, the average person must go on to utilize the long term energy source in the body - the fat. (fodsupport.org)

These are, in turn, transferred to the electron transport chain with the production of ATP. (medscape.com)
What happens if you lack the proteins to transport Acyl-Coa into the matrix? (flashcardmachine.com)

The program's functions include the initial screening of all newborns, identifying screen-positive newborns, diagnosing conditions, communicating with families, ensuring that affected children are referred to treatment centers, following up with long-term outcomes, and educating physicians and the public according to individual state or jurisdictional guidelines. (acog.org)

Anatomy5

Organisms1

Diseases2

Chemicals and Drugs83

Analytical, Diagnostic and Therapeutic Techniques and Equipment5

Phenomena and Processes13

Technology, Industry, Agriculture1

Information Science3

Deficiency42

Mitochondrial4

Carnitine2

Enoyl-CoA hydra1

LCHAD1

Coenzyme5

Hydroxyacyl-CoA dehydrogenase1

Enzyme7

Mitochondria3

Synthetase3

Acetyl CoA2

Gene2

Beta-oxidation1

Thioester1

Lyase1

Liver3

Defects2

Fatty acid1

Disorders3

ACADL1

MeSH1

Cardiac1

Medium-chain1

Accumulation1

Electron-transfer1

Enzymes1

Intermediate1

Carbon1

Specific1

Compound1

Quantitative1

Activity1

Transport2

Include1