产品名称 ACADVL Acyl-CoA Dehydrogenase, Very Long Chain Human Recombinant Protein 产品描述 ACADVL Human Recombinant produced in E.Coli is a non-glycosylated, polypeptide chain containing 636 amino acids (41-655 a.a.) and having a molecular mass of 68.5 kda. ACADVL contains 21 amino acid His-...
Protein Precursors geneticsA gene encoding the precursor for a novel member of the human acyl-CoA dehydrogenase (ACD) gene family has been isolated which maps to human chromosome 11q25. The cDNA contains an open reading frame of 1248 nucleotides encoding a predicted 415-amino-acid peptide, and ...
This review examines the structure of medium chain acyl-CoA dehydrogenase, as a representative of the dehydrogenase family, with respect to the catalytic mechanism and its broad chain length specificity. Comparing the structures of four other acyl-CoA dehydrogenases provides further insights into the ...
Vol. 1 No. 7 Medium chain acyl-CoA dehydrogenase deficiency: Human genome epidemiology review Sophia S. Wang, phDis2,P a u l M.Fernhofi MD', W. H a r r y Hannon, P~D*a, n d Muin J. Khoury, MD, phLI2 Medium chain acyl-CoA dehydrogenase (MCAD) is a tetrameric flavoprotein ...
Blots were probed with anti-GFP (Roche, #11814460001) and anti-glyceraldehyde 3-phosphat dehydrogenase (GAPDH; gift from Sepp Kohlwein, University of Graz) antibodies and the respective peroxidase-conjugated secondary antibodies (Sigma). Densitometric quantification was performed with Image Lab 5.2 ...
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is a common inborn error of mitochondrial fatty acid oxidation. To determine if immunoreactive enzyme protein is present in patients with MCAD deficiency, we studied cultured skin fibroblasts from patients with the 985 point mutation, present in ab...
Three proteins were found significantly regulated in both patient groups: adenylate kinase 4 (AK4), nucleoside diphosphate kinase A (NME1) and aldehyde dehydrogenase family 4 member A1 (ALDH4A1). Proteins AK4 and NME1 deserve further investigation because of their involvement in energy reprogramming...
(C6–C16) chain acyl-CoA specific proteins consisting of a homotetramer of 41–45 kDa subunits form an enzyme family located in the mitochondrial matrix. Very long-chain (C12–C24) acyl-CoA dehydrogenase is a homodimer of 71 kDa subunits and is located in mitochondrial inner membr...
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is the most common disorder of mitochondrial fatty acid β-oxidation and a target disease of newborn screening in many countries.We report on two siblings with mild MCAD deficiency associated with a novel splice site mutation in the ACADM gene...
Related to acyl CoA dehydrogenase deficiency:Carnitine deficiency ac·yl (ăs′əl) n.Chemistry An organic group having the general formula RCO-, derived from an organic acid. [ac(id)+-yl.] American Heritage® Dictionary of the English Language, Fifth Edition. Copyright © 2016 by Hough...