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The Biochemical Cause for the Defective Muscle Glycogen Synthesis - Essay Example

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The paper describes hypotonia, poor feeding. In an effort to diagnose his condition, you examined mitochondria in muscle cells obtained from a biopsy and detect a deficiency in oxidative metabolism. The patient’s mitochondria consumed oxygen normally when incubated with pyruvate and malate…
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The Biochemical Cause for the Defective Muscle Glycogen Synthesis
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Download file to see previous pages The lack of the desaturase enzyme results in a build-up of unreduced LFA and an increased request of carnitine to transport the accumulating waste products.  The carnitine is then becoming acylated with the  LFA which results in low serum carnitine and disruption of fatty acid metabolism overall. 2. Wakil’s pioneering studies on the synthesis of fatty acids included the crucial observation that bicarbonate was required for the synthesis of palmitoyl CoA. He was surprised to find that very low levels of bicarbonate could sustain palmitate biosynthesis and that there is no correlation between the amount of bicarbonate required and the amount of palmitate produced. Explain these observations and describe how radiolabelled bicarbonate could be used to support your explanation. The following reaction is the accepted mechanism for producing the long chain fatty acid palmitoyl-CoA.  When acetyl coenzyme A, malonyl coenzyme A and reduced triphosphopyridine nucleotide (TPNH) are used as substrates palmitic acid is the principal product of the reaction.  Naturally, the reaction is enzymatic. Obtained from metazoic and microbial cells. Wakil and Sterbi et al favoured a simple condensation that would yield an alpha-carboxyl-beta-ketothioester, whereas Vagelos and Alberts and Lynenet al have favoured a concerted condensation-decarboxylation that would yield a beta-ketothioester.  I dreamt about studying fatty acid synthesis in two different experimental systems; cultured rat hepatocytes and Escherichia coli.  ...Download file to see next pagesRead More
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