Succinyl-CoA:3-ketoacid coenzyme A transferase (SCOT): Development of an antibody to human SCOT and diagnostic use in hereditary SCOT deficiency
| Autor: | Toshiyuki Fukao, Ronald J.A. Wanders, Naomi Kondo, Sacha Kassovska-Bratinova, Haruo Shintaku, Ken Hirayama, Xiang-Qian Song, Tadao Orii, Grant A. Mitchell, Magdalena Ugarte, Perry Churchill, Hiroh Watanabe |
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| Přispěvatelé: | Faculteit der Geneeskunde |
| Rok vydání: | 1997 |
| Předmět: |
Blotting
Western Ketone Bodies Biology Lipid Metabolism Inborn Errors law.invention chemistry.chemical_compound law Coenzyme A transferase Immunoblot Analysis medicine Animals Humans Succinyl-CoA Fibroblast Molecular Biology Antibody Immunoassay chemistry.chemical_classification Ketone body Inborn error of metabolism Molecular biology Recombinant Proteins Enzyme assay Rats medicine.anatomical_structure Enzyme chemistry Biochemistry Recombinant DNA biology.protein Ketone bodies Molecular Medicine Coenzyme A-Transferases Immunoblot analysis |
| Zdroj: | Biochimica et Biophysica Acta, 1360, 151-156 |
| ISSN: | 0925-4439 0006-3002 |
| DOI: | 10.1016/s0925-4439(96)00074-9 |
| Popis: | Succinyl-CoA:3-ketoacid CoA transferase (SCOT) is a key enzyme for ketone body utilization. Hereditary SCOT deficiency in humans (McKusick catalogue number 245050) is characterized by intermittent ketoacidotic attacks and permanent hyperketonemia. Since previously-available antibody to rat SCOT did not crossreact with human SCOT, we developed an antibody against recombinant human SCOT expressed in a bacterial system. The recombinant SCOT was insoluble except under denaturing conditions. Antibody raised to this polypeptide recognized denatured SCOT and proved useful for immunoblot analysis. On immunoblots, SCOT was easily detectable in control fibroblasts and lymphocytes but was detected neither in fibroblast extracts from four SCOT-deficient patients, nor in lymphocytes from two SCOT-deficient patients. These data indicate that immunoblot analysis is useful for diagnosis of SCOT deficiency in combination with enzyme assay. |
| Databáze: | OpenAIRE |
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