• Carnitine binds acyl residues and helps in their elimination, decreasing the number of acyl residues conjugated with coenzyme A (CoA) and increasing the ratio between free and acylated CoA. (medscape.com)
  • The 3 areas of involvement include (1) the cardiac muscle, which is affected by progressive cardiomyopathy (by far, the most common form of presentation), (2) the CNS, which is affected by encephalopathy caused by hypoketotic hypoglycemia, and (3) the skeletal muscle, which is affected by myopathy. (medscape.com)
  • 6. Furuebisu, M., Deguchi, S. and Okuda, K. Identification of cortisone 5β-reductase as δ 4 -3-ketosteroid 5β-reductase. (qmul.ac.uk)
  • 7. Okuda, A. and Okuda, K. Purification and characterization of δ 4 -3-ketosteroid 5β-reductase. (qmul.ac.uk)
  • The bile acid intermediates 7α,12α-dihydroxy-4-cholesten-3-one and 7α-hydroxy-4-cholesten-3-one can also act as substrates . (qmul.ac.uk)
  • Regulation of the intramitochondrial free CoA also is affected, with accumulation of acyl-CoA esters in the mitochondria. (medscape.com)
  • A number of pharmaceutical steroid synthons are currently produced through the microbial side-chain cleavage of natural sterols as an alternative to multistep chemical synthesis. (cyberleninka.org)
  • 2012). These synthons can be produced by microbial side-chain cleavage of cholesterol or phytosterols as an alternative to multi-step chemical synthesis based on digoxigenin, a steroid found exclusively in the flowers and leaves of Digitalis plants, as a starting material. (cyberleninka.org)
  • 3. Levy, H.R. and Talalay, P. Enzymatic introduction of double bonds into steroid ring A. (qmul.ac.uk)
  • It plays an essential role in the transfer of long-chain fatty acids into the mitochondria for beta-oxidation. (medscape.com)
  • Consequently, long-chain fatty acids are not available for beta-oxidation and energy production, and the production of ketone bodies (which are used by the brain) is also impaired. (medscape.com)
  • Therefore, we have constructed a MSMEG_6039 (kshB1) gene deletion mutant of M. smegmatis MS6039 that transforms efficiently natural sterols (e.g. cholesterol and phytos-terols) into 1,4-androstadiene-3,17-dione. (cyberleninka.org)
  • In addition, we have demonstrated that a double deletion mutant M. smegmatis MS6039-5941 [DMSMEG_6039 (DkshB1) and DMSMEG_5941 (DkstD1)] transforms natural sterols into 4-androstene-3,17-dione with high yields. (cyberleninka.org)