Cyclisation onto the acyl side chain
Structure and function of a single-chain, multi-domain long-chain acyl-CoA carboxylase
... long-chain acyl-CoA substrates, although it is also active toward short- and medium-chain acyl-CoAs, and we have named it long-chain acyl-CoA carboxylase ...
27
Arabidopsis acyl-acyl carrier protein synthetase AAE15 with medium chain fatty acid specificity is functional in cyanobacteria
... the acyl activating enzyme AAE15 of Arabidopsis encoded by At4g14070, which is a homologue of a cyanobacterial acyl-ACP synthetase ...medium chain fatty acid (C10–C14) substrates in ...medium ...
9
Fatty acyl chain composition in the determination of renal membrane order
... fatty acyl chain length and phospholipid fatty acyl chain unsaturation in the determination of rat renal brush border membrane order were examined using multilamellar ...
7
Child Neurology: Medium-chain acyl-coenzyme A dehydrogenase deficiency
... Medium-chain acyl-coenzyme A dehydrogenase deficiency Medium-chain acyl-coenzyme A dehydrogenase (MCAD) deficiency (MCADD) is an autosomal recessive inherited fatty acid oxidation ...medium- ...
5
Targeting Long Chain Acyl-CoA Synthetases for Cancer Therapy.
... long-chain acyl-CoA synthetases, which are enzymes that catalyze the activation of long-chain fatty acids of 12–22 ...long-chain acyl-CoA synthetases are deregulated in many types of ...
16
Identification of two variant short chain acyl coenzyme A dehydrogenase alleles, each containing a different point mutation in a patient with short chain acyl coenzyme A dehydrogenase deficiency
... short chain acyl-CoA dehydrogenase (SCAD) gene were identified in a SCAD-deficient patient (YH2065) using the polymerase chain reaction to amplify cDNA synthesized from total RNA from her ...
9
Screening for medium-chain acyl CoA dehydrogenase deficiency: current perspectives
... Dovepress Screening for medium-chain acyl CoA dehydrogenase deficiency or severe), hypoglycemia, and documented liver disease. The biochemical diagnosis is based on plasma acylcarnitine profile ...
10
Substrate Specificity of Microsomal -Acyl-sn-glycero- -phosphocholine Acyltransferase in Rat Salivary Glands for Polyunsaturated Long-Chain Acyl-CoAs
... an acyl chain length of more than１ ８carbon atoms and more than３-５double bonds in the acyl ...eicosanoid-related acyl-CoAs are a suitable substrate for１-acyl-sn-glycero-３-phosphocholine ...
5
Parental experiences of raising a child with medium chain Acyl CoA Dehydrogenase deficiency
... Abstract Newborn screening enabling early diagnosis of medium chain acyl-CoA dehydrogenase deficiency (MCADD) has dramatically improved health outcomes in children with MCADD. Achieving those outcomes ...
9
In vitro and in vivo consequences of variant medium-chain acyl-CoA dehydrogenase genotypes
... In In The online metabolic and molecular bases of inherited disease. 8th edition. Edited by Valle D, Scriver CR. New York: McGraw-Hill; 2001. 2. Touw CM, Smit GP, de Vries M, de Klerk JB, Bosch AM, Visser G, Mulder MF, ...
8
Medium-chain Acyl-CoA dehydrogenase deficiency presenting with neonatal pulmonary haemorrhage
... Medium-chain Acyl-CoA dehydrogenase deficiency (MCADD) is the most common inherited disorder of mitochondrial fatty acid beta-oxidation. The overall frequency of the disorder is highest in northern Europe ...
6
Prolonged QTc Interval in Association With Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency
... fi common disorder of fatty acid oxida- tion, with an incidence of 1 in 15 000 to 1 in 20 000 births. MCAD de fi ciency is an autosomal recessive disorder char- acterized by a defect in mitochondrial fatty acid b ...
8
Spectrum of Medium-Chain Acyl-CoA Dehydrogenase Deficiency Detected by Newborn Screening
... M EDIUM-CHAIN ACYL-CoA DEHYDROGENASE (MCAD) deficiency is the most common fatty acid oxidation disorder, occurring in ⬃ 1:15 000 births. 1 The MCAD protein is an enzyme that catalyzes the breakdown of fatty ...
9
Characterization of acyl chain position in unsaturated phosphatidylcholines using differential mobility-mass spectrometry
... of acyl chains on the glycerol backbone may be required for the protein to function in steroid synthesis ...the acyl chain composition and glycerol backbone position of the target ...specific ...
37
Acyl chain elongation drives ketosynthase substrate selectivity in trans acyltransferase polyketide synthases
... trans-acyl transferase polyketide synthases Matthew Jenner [a] , José Pedro Afonso [a] , Hannah R. Bailey [a] , Sarah Frank [b] , Annette Kampa [b] , Jörn Piel* [b] and Neil J. Oldham* [a] Abstract: Type I modular ...
6
Hepatocyte Elovl6 determines ceramide acyl-chain length and hepatic insulin sensitivity in mice
... the acyl-chain composition of other lipids that regulate hepatic insulin sensitivity given that our lipidomic platform did not determine the fatty acyl constituents, positional isomers, or double ...
148
Familial Reye-Like Syndrome: A Presentation of Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency
... Medium-chain acyl-coenzyme A (CoA) dehydro- genase deficiency is a recently described, autosomal recessive disorder of fatty acid oxidation that has a superficial similarity to Reye synd[r] ...
6
Recurrent Myoglobinuria as a Presenting Manifestation of Very Long Chain Acyl Coenzyme A Dehydrogenase Deficiency
... Mitochondrial very long chain acyl-coA dehydrogenase deficiency: identification of this new inborn error of fatty oxidation in 11 patients.. Presented at the Society for the Study of Inb[r] ...
5
Short-Chain Acyl-CoA Dehydrogenase Deficiency: Studies in a Large Family Adding to the Complexity of the Disorder
... electrospray tandem mass spectrometry. 9 The activity of short- chain acyl-CoA dehydrogenase in lymphocytes or fibroblasts was measured using a high-performance liquid chromatography method. Cell lysates ...
6
On the Calculation of Acyl Chain Order Parameters from Lipid Simulations
... sn-2 chain for the GROMOS-CKP force field calculated from the POPC simulations performed in our previous comparative force field work 2 , using both the NMRlipids analysis method and the modified version of ...
12