Michael J. Bennett, PhD - AACC.org
... the fatal clinical phenotype and the first to identify neonatal metabolite abnormalities in medium-chain acyl-CoA dehydrogenase ... He is currently studying the hyperinsulinism associated with deficiency of short-chain L-3-hydroxyacyl-CoA dehydrogenase (SCHAD ...https://www.aacc.org/Community/Awards/Hall-of-Fame/Bios/L-to-S/Michael-Bennett.aspx
Genetic Information Center Inborn erros of metabolism 3-HYDROXYACYL-CoA DEHYDROGENASE DEFICIENCY (SCHAD) HADH DEFICIENCY, SCHAD ... Short-chain 3-hydroxyacyl-CoA dehydrogenase. 2.48. 5.10. 8.70. -Ámol/min/g tissue. muscle. ... 3-Hydroxybutyric acid. 0.00. 0.00. 0.00. 3.00. increased. urine. childhood 6-12y. ... short-chain L-3-hydroxyacyl-CoA (SCHAD, HADH) deficiency is characterised by hypoglycemia with hyperinsulinism in the neonatal ...http://www.metagene.de/disease/3HYDROXYACYLCoADEHYDROGENASEDEFICIENCYSCHAD_393.html
Fatty acid metabolism - Wikipedia
Dehydrogenation by acyl-CoA dehydrogenase, yielding 1 FADH2 Hydration by enoyl-CoA hydratase Dehydrogenation by 3-hydroxyacyl- ... Acetyl-CoA is formed into malonyl-CoA by acetyl-CoA carboxylase, at which point malonyl-CoA is destined to feed into the fatty ... CoA dehydrogenase, yielding 1 NADH + H+ Cleavage by thiolase, yielding 1 acetyl-CoA and a fatty acid that has now been ... acetyl-CoA and 1 molecule of propionyl-CoA per molecule of fatty acid. Each beta oxidative cut of the acyl-CoA molecule yields ...https://en.wikipedia.org/wiki/Fatty_acid_metabolism
Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
HIV-1 Vpr regulates mitochondrial respiration and enhances the activity of hydroxyacyl-CoA dehydrogenase (HADH) through ... NAD+ + (S)-3-Hydroxy-2-methyl-butanoyl-CoA = NADH + 2-Methyl-3-acetoacetyl-CoA ( Valine,Leucine and Isoleucine degradation ) 4 ... NAD+ + (S)-3-Hydroxy-butanoyl-CoA = NADH + Acetoacetyl-CoA ( Butanoate metabolism ) 4 ... NAD+ + (S)-3-Hydroxy-butanoyl-CoA = NADH + Acetoacetyl-CoA ( Tryptophan degradation ) 4 ...https://pharos.nih.gov/idg/targets/Q16836
Acyl-CoA dehydrogenase, very long chain, deficiency of (Symptoms, signs, causes, treatments & definition) - Medigest
Medigest has all you need to know about Acyl-CoA dehydrogenase, very long chain, deficiency of - Symptoms and Signs, Causes, ... Looking for information on Acyl-CoA dehydrogenase, very long chain, deficiency of? ... Acyl-CoA dehydrogenase, very long chain, deficiency of Below you will find more information about Acyl-CoA dehydrogenase, very ... Discuss Acyl-CoA dehydrogenase, very long chain, deficiency of in our forums Discuss Acyl-CoA dehydrogenase, very long chain, ...https://www.medigest.uk/diseases/acyl-coa-dehydrogenase-very-long-chain-deficiency-of/
3-hydroxyacyl-CoA dehydrogenase - Wikipedia
L-hydroxyacyl-CoA dehydrogenase 3beta-hydroxyacyl coenzyme A dehydrogenase beta-hydroxy acid dehydrogenase beta-hydroxyacyl CoA ... dehydrogenase beta-hydroxyacyl dehydrogenase beta-hydroxyacyl-coenzyme A synthetase beta-hydroxyacylcoenzyme A dehydrogenase ... beta-Hydroxyacyl coenzyme A dehydrogenase". J. Biol. Chem. 207 (2): 631-8. PMID 13163047. Molecular and Cellular Biology portal ... Hydroxyacyl-Coenzyme A dehydrogenase HSD17B10 - 3-Hydroxyacyl-CoA dehydrogenase type-2 EHHADH - Peroxisomal bifunctional enzyme ...https://en.wikipedia.org/wiki/3-hydroxyacyl-CoA_dehydrogenase
Human Metabolome Database: Showing Protein 3-hydroxyacyl-CoA dehydrogenase type-2 (HMDBP00379)
2-Methylacetoacetyl-CoA + NADH. details 2-Methyl-3-hydroxybutyryl-CoA + NAD → 2-Methylacetoacetyl-CoA + NADH + Hydrogen Ion. ... Submicroscopic duplications of the hydroxysteroid dehydrogenase HSD17B10 and the E3 ubiquitin ligase HUWE1 are associated with ... 2004 Sep 17;342(3):943-52. [PubMed:15342248 ] *Lustbader JW, Cirilli M, Lin C, Xu HW, Takuma K, Wang N, Caspersen C, Chen X, ... S)-3-hydroxyacyl-CoA + NAD → 3-oxoacyl-CoA + NADH. details 2-Methyl-3-hydroxybutyryl-CoA + NAD → ...http://www.hmdb.ca/proteins/HMDBP00379
3-hydroxyacyl-CoA dehydrogenase | definition of 3-hydroxyacyl-CoA dehydrogenase by Medical dictionary
What is 3-hydroxyacyl-CoA dehydrogenase? Meaning of 3-hydroxyacyl-CoA dehydrogenase medical term. What does 3-hydroxyacyl-CoA ... Looking for online definition of 3-hydroxyacyl-CoA dehydrogenase in the Medical Dictionary? 3-hydroxyacyl-CoA dehydrogenase ... yl-CoA de·hy·dro·gen·ase. (hī-drok'sē-as'il dē'hī-drō'jen-ā), β-Hydroxyacyl dehydrogenase; an enzyme catalyzing the oxidation ... 3-hydroxyacyl-CoA dehydrogenase. Also found in: Acronyms, Wikipedia. 3-hy·drox·y·ac· ...http://medical-dictionary.thefreedictionary.com/3-hydroxyacyl-CoA+dehydrogenase
Structure Cluster - 1E3W: Rat brain 3-hydroxyacyl-CoA dehydrogenase binary complex with NADH and 3-keto butyrate 3D...
Recognition of Structurally Diverse Substrates by Type II 3-Hydroxyacyl-Coa Dehydrogenase (Hadh II) Amyloid-Beta Binding ... 3] Andreeva A., Howorth D., Chandonia J.-M., Brenner S.E., Hubbard T.J.P., Chothia C., Murzin A.G. (2008).. Data growth and its ... Rat brain 3-hydroxyacyl-CoA dehydrogenase binary complex with NADH and 3-keto butyrate. ... If available, the SCOP 1.75 domain assignment  is used. Otherwise algorithmic domain assignments are computed using the ...http://www.rcsb.org/pdb/explore/structureCluster.do?structureId=1E3W
Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD) | CHEO NSO
Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD)Download version for offline viewing or printing. [463.65kB] ... Babies with the diseases Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD) or Trifunctional Protein Deficiency (TFP ...https://www.newbornscreening.on.ca/en/node/197
Ocular characteristics in 10 children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency : a cross-sectional study with...
86, nr 3, 329-337 s. Nyckelord [en] Chorioretinal atrophy, electroetinography, LCHAD deficiency, myopia Nationell ämneskategori ... 86, nr 3, 329-337 s.Artikel i tidskrift (Refereegranskat) Published Abstract [en] PURPOSE ... Ocular characteristics in 10 children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: a cross-sectional study with ... To present long-term ocular complications and electroretinographic (ERG) findings in children with long-chain 3-hydroxyacyl-CoA ...http://uu.diva-portal.org/smash/record.jsf?faces-redirect=true&language=sv&searchType=SIMPLE&query=&af=%5B%5D&aq=%5B%5B%5D%5D&aq2=%5B%5B%5D%5D&aqe=%5B%5D&pid=diva2%3A220279&noOfRows=50&sortOrder=author_sort_asc&sortOrder2=title_sort_asc&onlyFullText=false&sf=all
Fatal pitfalls in newborn screening for mitochondrial trifunctional protein (MTP)/long-chain 3-Hydroxyacyl-CoA dehydrogenase ...
... and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency are long-chain fatty acid oxidation disorders with ... For very-long-chain acyl-CoA dehydrogenase deficiency (VLCADD), the most common long-chain fatty oxidation defect, it has been ... Ficicioglu C, Coughlin CR 2nd, Bennett MJ, Yudkoff M. Very long-chain acyl-CoA dehydrogenase deficiency in a patient with ... A near-miss: very long chain acyl-CoA dehydrogenase deficiency with normal primary markers in the initial well-timed newborn ...https://ojrd.biomedcentral.com/articles/10.1186/s13023-018-0875-6
LCHAD (Long-Chain 3-Hydroxyacyl CoA Dehydrogenase) deficiency
The other 2 activities of the protein are 2-enoyl coenzyme A (CoA) hydratase (LCEH) and long-chain 3-ketoacyl CoA thiolase ( ... 2002). Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: clinical presentation and follow-up of 50 patients. Pediatrics. ... LCHAD (Long-Chain 3-Hydroxyacyl CoA Dehydrogenase) deficiency. LCHAD (long-chain 3-hydroxyacyl CoA dehydrogenase) deficiency is ... Chronically 3-hydroxylated dicarboxylic acids and nonhydroxylated dicarboxylic acids will be positive. Plasma carnitine may be ...http://www.brainology.net/neurology/lchad.html
Medium chain acyl CoA dehydrogenase deficiency (MCAD), Short chain acyl CoA dehydrogenase deficiency (SCAD) ... Medium chain acyl CoA dehydrogenase deficiency (MCAD), Short chain acyl CoA dehydrogenase deficiency (SCAD) ... Medium chain acyl CoA dehydrogenase deficiency (MCAD), Short chain acyl CoA dehydrogenase deficiency (SCAD) ... Long chain/very long chain acyl CoA dehydrogenase deficiency, ... Long chain/very long chain acyl CoA dehydrogenase deficiency, ...https://www.genedx.com/test-catalog/disorders/long-chain-3-hydroxyacyl-coa-dehydrogenase-deficiency-lchad/
Search of: 'MERRF Syndrome' OR 'Mitochondrial Myopathies' OR 'myoclonic epilepsy with ragged-red fibers' - List Results -...
CoA) Dehydrogenase (LCHAD) Associated Neuropathy. *Peripheral Neuropathy ... 3 Years to 65 Years (Child, Adult). NCT01339494. H-24783. July 2009. April 2016. April 2016. April 20, 2011. April 5, 2016. * ... and 3 more...). 202. All. 16 Years to 80 Years (Child, Adult, Senior). NCT03323749. SPIMM-301. MMPOWER-3. October 27, 2017. ... and 3 more...). 300. All. 4 Years and older (Child, Adult, Senior). NCT01532791. AAAB1425. 5P01HD032062. July 2004. January ...https://clinicaltrials.gov/ct2/results?cond=%22MERRF+Syndrome%22+OR+%22Mitochondrial+Myopathies%22+OR+%22myoclonic+epilepsy+with+ragged-red+fibers%22&show_rss=Y&sel_rss=mod14
EHHADH Gene - GeneCards | ECHP Protein | ECHP Antibody
Enoyl-CoA Hydratase And 3-Hydroxyacyl CoA Dehydrogenase, including: function, proteins, disorders, pathways, orthologs, and ... Enoyl-CoA Hydratase And 3-Hydroxyacyl CoA Dehydrogenase 2 3 5 * Enoyl-Coenzyme A, Hydratase/3-Hydroxyacyl Coenzyme A ... EHHADH (Enoyl-CoA Hydratase And 3-Hydroxyacyl CoA Dehydrogenase) is a Protein Coding gene. Diseases associated with EHHADH ... The GeneCards human gene database index: 2 3 5 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z ...https://www.genecards.org/cgi-bin/carddisp.pl?gene=EHHADH
Ear canal inflammation and Earlobe itch and Fainting and Impaired speech articulation - Symptom Checker - check medical...
2. 2-methylbutyryl-coenzyme A dehydrogenase deficiency. 3. 3 alpha methylcrotonyl-Coa carboxylase 1 deficiency. 4. 3 alpha ... methylcrotonyl-coa carboxylase 2 deficiency. 5. 3-alpha-Hydroxyacyl-CoA Dehydrogenase Deficiency. 6. 3-alpha-hydroxyacyl- ... coenzyme A dehydrogenase deficiency. 7. 3-methylcrotonyl-CoA carboxylase deficiency. 8. Abdominal Aneurysm. 9. ...https://wrongdiagnosis.com/cosymptoms/ear-canal-inflammation/earlobe-itch/fainting/impaired-speech-articulation.htm
Chronic fibromyalgia-like symptoms and Fatigue and Gait disorder and Muscle aches and Stiffness - Symptom Checker - check...
2-methylbutyryl-coenzyme A dehydrogenase deficiency. 4. 3 alpha methylcrotonyl-Coa carboxylase 1 deficiency. 5. 3 alpha ... methylcrotonyl-coa carboxylase 2 deficiency. 6. 3-alpha-Hydroxyacyl-CoA Dehydrogenase Deficiency. 7. 3-alpha-hydroxyacyl- ... coenzyme A dehydrogenase deficiency. 8. 3-methylcrotonyl-CoA carboxylase deficiency. 9. Abdominal Cancer. 10. Abdominal abscess ...http://wrongdiagnosis.com/cosymptoms/chronic-fibromyalgia-like-symptoms/fatigue/gait-disorders/muscle-aches/stiffness.htm
Anti-Alcohol Dehydrogenase 2 Antibody Products | Biocompare.com
Compare Anti-Alcohol Dehydrogenase 2 Antibody Products from leading suppliers on Biocompare. View specifications, prices, ... Rabbit anti-human zinc binding alcohol dehydrogenase domain containing 2 polyclonal Antibody ...https://www.biocompare.com/pfu/110447/soids/144790/Antibodies/Alcohol_Dehydrogenase_2
3-hydroxyacyl-coenzyme A dehydrogenase deficiency - Wikipedia
People with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency have inadequate levels of an enzyme required for a step that ... Problems related to 3-hydroxyacyl-coenzyme A dehydrogenase deficiency can be triggered by periods of fasting or by illnesses ... 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (HADH deficiency) is a rare condition that prevents the body from converting ... Individuals with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency are also at risk for complications such as seizures, life- ...https://en.wikipedia.org/wiki/3-hydroxyacyl-coenzyme_A_dehydrogenase_deficiency
Prevalence of Causes of Symptom: Reduced muscle tone - RightDiagnosis.com
Hydroxyacyl-coa dehydrogenase, type 2, deficiency... reduced muscle tone *Hyperdibasic aminoaciduria type 2... reduced muscle ... Very Long Chain Acyl CoA Dehydrogenase Deficiency -- adult-onset... reduced muscle tone *Very Long Chain Acyl CoA Dehydrogenase ... Short Chain Acyl CoA Dehydrogenase Deficiency (SCAD)... reduced muscle tone *Short limb dwarf lethal, Mcalister Crane type... ... Acyl-CoA dehydrogenase, short chain, deficiency of... reduced muscle tone *Adducted thumb syndrome recessive form... reduced ...https://www.rightdiagnosis.com/symptoms/reduced_muscle_tone.htm
Idiopathic Recurrent Pericarditis disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials
3. adipose tissue. MP:0005375 9.67. ADIPOQ LEP RETN TNFRSF1A 4. immune system. MP:0005387 9.63. NAMPT RETN TNFRSF1A ADIPOQ LEP ... 3. Refractory idiopathic recurrent pericarditis: treatment with interleukin-1 receptor antagonist is an option! ( 28746150 ) ...https://www.malacards.org/card/idiopathic_recurrent_pericarditis
Fatty acid oxidation complex, alpha subunit, mitochondrial (IPR012803) | InterPro | EMBL-EBI
GO:0004300 enoyl-CoA hydratase activity Cellular Component. GO:0016507 mitochondrial fatty acid beta-oxidation multienzyme ... Enoyl-CoA hydratase/isomerase (IPR001753) *Fatty acid oxidation complex, alpha subunit, mitochondrial (IPR012803) ... Subunit activities include: enoyl-CoA hydratase (EC:184.108.40.206) and 3-hydroxyacyl-CoA dehydrogenase (EC:220.127.116.11). Some ... The beta subunit has acetyl-CoA C-acyltransferase (EC:18.104.22.168) activity. ...http://www.ebi.ac.uk/interpro/entry/IPR012803
LIPID MAPS Lipidomics Gateway
enoyl-CoA hydratase/epimerase and isomerase/3-hydroxyacyl-CoA dehydrogenase [Escherichia coli str. K-12 substr. MG1655]. ...http://lipidmaps.org/data/proteome/LMPD_detail.php?LMP_ID=LMP007603
CPT1a-Dependent Long-Chain Fatty Acid Oxidation Contributes to Maintaining Glucagon Secretion from Pancreatic Islets.
Acyl-coa Dehydrogenase, Long-chain. A flavoprotein oxidoreductase that has specificity for long-chain fatty acids. It forms a ... Acetyl-coa C-acyltransferase. Enzyme that catalyzes the final step of fatty acid oxidation in which ACETYL COA is released and ... 3-hydroxyacyl Coa Dehydrogenases. Enzymes that reversibly catalyze the oxidation of a 3-hydroxyacyl CoA to 3-ketoacyl CoA in ... the CoA ester of a fatty acid two carbons shorter is formed. ... are based on 3 strategies: - limitation of lipid intake in the ...https://www.bioportfolio.com/resources/pmarticle/2069128/CPT1a-Dependent-Long-Chain-Fatty-Acid-Oxidation-Contributes-to-Maintaining-Glucagon-Secretion.html