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. 1966 Jul;56(1):236–242. doi: 10.1073/pnas.56.1.236

Isovaleric acidemia: a new genetic defect of leucine metabolism.

K Tanaka, M A Budd, M L Efron, K J Isselbacher
PMCID: PMC285701  PMID: 5229850

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. AHMED K., SCHOLEFIELD P. G. Studies on fatty acid oxidation. 8. The effects of fatty acids on metabolism of rat-brain cortex in vitro. Biochem J. 1961 Oct;81:45–53. doi: 10.1042/bj0810045. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. ANNISON E. F. Studies on the volatile fatty acids of sheep blood with special reference to formic acid. Biochem J. 1954 Dec;58(4):670–680. doi: 10.1042/bj0580670. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. BACHHAWAT B. K., ROBINSON W. G., COON M. J. Enzymatic carboxylation of beta-hydroxyisovaleryl coenzyme A. J Biol Chem. 1956 Apr;219(2):539–550. [PubMed] [Google Scholar]
  4. BRIN M., YONEMOTO R. H. Stimulation of the glucose oxidative pathway in human erythrocytes by methylene blue. J Biol Chem. 1958 Jan;230(1):307–317. [PubMed] [Google Scholar]
  5. CAVALLINI D., FRONTALI N. Quantitative determination of keto-acids by paper partition chromatography. Biochim Biophys Acta. 1954 Mar;13(3):439–445. doi: 10.1016/0006-3002(54)90351-0. [DOI] [PubMed] [Google Scholar]
  6. COCHRANE W. A., PAYNE W. W., SIMPKISS M. J., WOOLF L. I. Familial hypoglycemia precipitated by amino acids. J Clin Invest. 1956 Apr;35(4):411–422. doi: 10.1172/JCI103292. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. DAHL D. R., DAHL N., SAMSON F. E., Jr A study on the narcotic action of the short chain fatty acids. J Clin Invest. 1956 Nov;35(11):1291–1298. doi: 10.1172/JCI103384. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. DEL CAMPILLO-CAMPBELL A., DEKKER E. E., COON M. J. Carboxylation of beta-methylcrotonyl coenzyme A by a purified enzyme from chicken liver. Biochim Biophys Acta. 1959 Jan;31(1):290–292. doi: 10.1016/0006-3002(59)90483-4. [DOI] [PubMed] [Google Scholar]
  9. EFRON M. L. Two-way separation of amino acids and other ninhydrin-reacting substances by high-voltage electrophoresis followed by paper chromatography. Biochem J. 1959 Aug;72:691–694. doi: 10.1042/bj0720691. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. FISCHER J. E. Metabolism of beta-methyl-C5 fatty acids by mitochondria of rat liver: effect of biotin nutriture. Proc Soc Exp Biol Med. 1955 Feb;88(2):227–230. doi: 10.3181/00379727-88-21546. [DOI] [PubMed] [Google Scholar]
  11. GREEN D. E., MII S., MAHLER H. R., BOCK R. M. Studies on the fatty acid oxidizing system of animal tissues. III. Butyryl coenzyme A dehydrogenase. J Biol Chem. 1954 Jan;206(1):1–12. [PubMed] [Google Scholar]
  12. Hird F. J., Weidemann M. J. Oxidative phosphorylation accompanying oxidation of short-chain fatty acids by rat-liver mitochondria. Biochem J. 1966 Feb;98(2):378–388. doi: 10.1042/bj0980378. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. SAMSON F. E., Jr, WHITE R. P. Effects of fatty acid anions on the electroencephalogram of unanesthetized rabbits. Am J Physiol. 1956 Aug;186(2):271–274. doi: 10.1152/ajplegacy.1956.186.2.271. [DOI] [PubMed] [Google Scholar]
  14. SKOOG W. A., BECK W. S. Studies on the fibrinogen, dextran and phytohemagglutinin methods of isolating leukocytes. Blood. 1956 May;11(5):436–454. [PubMed] [Google Scholar]
  15. ZBOROWSKI J., WOJTCZAK L. INDUCTION OF SWELLING OF LIVER MITOCHONDRIA BY FATTY ACIDS OF VARIOUS CHAIN LENGTH. Biochim Biophys Acta. 1963 Oct 22;70:596–598. doi: 10.1016/0006-3002(63)90799-6. [DOI] [PubMed] [Google Scholar]

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