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Glucose, Fats and Energy• Glucose is a primary fuel– Glycogen reserves are exhausted in 24 – 48 h– Additional glucose comes from gluconeogenesis• Occurs concurrently with glycogenolysis, but also on its own when glycogenexhausted• Gluconeogenesis from pyruvate (via oxaloacetate) provides glucose toorgans that cannot use other fuels• Fatty acid oxidation provides alternative source of ATP, andfuel (ketones) to some other organs– Fatty acids are better fuels than amino acids and carbohydrates,• 1 g fat generates 37.7 kJ• 1 g carbohydrate generates 16.7 kJ– Energy may be used directly (heat) or stored chemically– Also promotes gluconeogenesis
Catabolism: A Bird’s-eye ViewProteinsGlycogen & starchTriacylglycerolsAmino acidsGlucoseFatty acidsATPPyruvateCO 2NH 4+Acetyl-CoAOxaloacetateTCA cycleGTPCO 2Acetyl CoA common intermediate – feedsinto TCA to complete oxidation processAbundance of acetyl-CoA stimulatesgluconeogenesisNADHQH 2Energy produced by catabolism stored asATPO 2ATP, GTP, NADH, QH 2H 2 OAfter Horton et al. Principles of Biochemistry 3e
Page 1 and 2: Acylcarnitines And InheritedMetabol
Page 3: CarnitineCOO -HO• Essential compo
Page 7 and 8: The Carnitine CycleFree carnitineFa
Page 9 and 10: Acyl Carnitines in Fatty AcidOxidat
Page 11 and 12: Blood spotsPlasma (and other fluids
Page 13 and 14: MS/MS 2• Parents of m/z 85 - acyl
Page 15 and 16: Normal Acylcarnitine Pattern16Apr00
Page 17 and 18: Why Numbers?• Acylcarnitines are
Page 20 and 21: CPT-I Deficiency 1• Blocked forma
Page 23: CPT-II Deficiency• Defect in rege
Page 29 and 30: Isovaleric AciduriaIM D 252 1 (1.02
Page 31 and 32: Glutaric Aciduria Type 1Tryptophans
Page 33 and 34: Worked Example 130Jan003IMD025 1 (1
Page 35 and 36: Worked Example 2010202-15201Feb02-1
Page 37 and 38: • Presentation- Very heterogeneou
Page 39 and 40: Medium Chain Acyl-CoA Dehydrogenase
Page 41 and 42: Worked Example 421Feb003IMD034 1 (1
Page 43 and 44: • Variable presentationPost-Morte
Page 45 and 46: Diagnosis?302459260263347456
Page 47 and 48: Why?• PAR 85 experiment not speci
Page 49 and 50: IsomersH 3 CH 2 C CH 2H 2 C CH 2H 3
Page 51 and 52: • Non-derivitisationOther Conside

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