Notes in d. Energy Metabolism III

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Published	07/28/2024	The largest concentration of glycogen are found stored in the {{c1::liver}} and {{c2::muscles}}
Published	07/28/2024	The reducing ends of glycogen are covalently attached to the protein {{c1::glycogenin}} and are the essential {{c1::core}} for glycogen synthesis…
Published	07/28/2024	Glucose-6-phosphate is converted to glucose-1-phosphate by {{c1::phosphoglucomutase}} which occurs in the {{c1::liver}} to maintain blood glucose…
Published	07/28/2024	Glucose-1-phosphate is converted to UDP-glucose by {{c1::uridylyltransferase}}
Published	07/28/2024	UDP glucose is converted into the glycogen by the enzyme {{c1::glycogen synthase}}
Published	07/28/2024	How much energy is used up in adding 1 glucose molecule to glycogen?
Published	07/28/2024	{{c1::Branching enzyme}} is responsible for creating the branches found on glycogen via an alpha 1-6 linkage
Published	07/28/2024	Glycogen breakdown is catalyzed by {{c1::glycogen phosphorylase}}, which produces {{c2::glucose-1-phosphate}}
Published	07/28/2024	Glycogen side chains are broken down by {{c1::glycogen debranching enzyme}} and the removed glucose will need to be phosphorylated before partici…
Published	07/28/2024	Skeletal muscle cannot contribute glucose to maintaining blood glucose levels because it lacks the enzyme {{c1::glucose-6-phosphatase}}
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Published	07/28/2024	{{c1::beta oxidation}} is the primary pathway of fatty acid catabolism resulting in {{c1::acetyl CoA}} and occurs in {{c1::mitochondrial matrix}}. Ace…
Published	07/28/2024	In a fasting state, {{c1::epinepherine/norepinepherine}} cause activation of {{c1::hormone-sensitive}} lipase
Published	07/28/2024	Free fatty acids circulate bound to {{c1::serum albumin}} and are taken up by the organs throughout the body
Published	07/28/2024	Fatty acids 12-carbons and longer use {{c1::carnitine palimtoyl transferase I and II}} as a carrier to enter the mitochondria (shorter fatty acid…
Published	07/28/2024	{{c1::Primary}} carnitine deficiencies are caused by mutations in carnitine {{c1::transporters}} which causes {{c1::cardiomyopathy}} because heart can…
Published	07/28/2024	{{c1::Secondary}} carnitine deficiencies are caused by accumulation of {{c1::acyl carnitines}} and {{c1::hemodialysis}} which causes {{c1::cardiomyopa…
Published	07/28/2024	The first enzyme in a beta oxidation cycle is {{c1::acyl CoA dehydrogenase}}
Published	07/28/2024	Propionyl CoA is a {{c1::three}} carbon fatty acyl chain produced by beta oxidation that is metabolized into {{c1::succinyl CoA }} in a three ste…
Published	07/28/2024	The {{c1::SCAD}} is the Acyl-CoA dehydrogenase that is used in beta oxidation of 2-4C fatty acids. Deficiency in SCAD is an {{c1::autosomal reces…
Published	07/28/2024	The {{c1::Medium chain acyl-coa dehydrogenase (MCAD)}} is the Acyl-CoA dehydrogenase that is used in beta oxidation of 4-12C fatty acids and defi…
Published	07/28/2024	The {{c1::VLCAD}} is the Acyl-CoA dehydrogenase that is used in beta oxidation of >20C fatty acids
Published	07/28/2024	Very long chain fatty acids can undergo beta oxidation in the {{c1::peroxisome}}
Published	07/28/2024	{{c2::Acyl-CoA oxidase}} is utilized in {{c1::peroxisomal}} beta oxidation and is involved in the production of {{c2::H202}}
Published	07/28/2024	Zellweger syndrome is a deficiency in {{c1::peroxisome FA oxidation}}
Published	07/28/2024	{{c1::X-linked adrenoleukodystrophy}} is a defect in import of {{c1::very long chain fatty acids}} into {{c2::peroxisomes}} leads to {{c1::a…
Published	07/28/2024	Refsum disease is a recessively inherited defect of {{c1::alpha-oxidation}} which is required to metabolize the highly branched {{c1::phytanic}} …
Published	07/28/2024	Ketone bodies are synthesized from {{c1::acetyl CoA}}
Published	07/28/2024	Insulin {{c1::promotes}} fatty acid biosynthesis
Published	07/28/2024	Glucagon {{c1::inhibits}} fatty acid biosynthesis
Published	07/28/2024	First {{c1::commited}} step of fatty acid synthesis is conversion of acetyl CoA to {{c1::Malonyl CoA}} which {{c2::inhibits}} transfer of Fatty acids …
Published	07/28/2024	{{c1::Phosphocreatine}} is a {{c1::short}} term reserve of ATP in muscles that can nonenzymatically breakdown to generate {{c2::creatinine}}, whi…
Published	07/28/2024	Von Gierke is type {{c1::I}} glycogen storage disease where there is a deficiency in glucose 6-{{c1::phosphatase (type 1A)}} which causes glucose to b…
Published	07/28/2024	Pompe type {{c1::II}} is a very rare glycogen storage disease that is caused by a deficiency in {{c1::lysosomal acid alpha-glucosidase}} which causes …
Published	07/28/2024	Cori is a type {{c1::III}} glycogen storage disease caused by deficiency in glycogen {{c1::debranching}} enzyme which usually manifests first with hyp…
Published	07/28/2024	Triacylglycerol-based stores are {{c1::high}} in energy content because fats are so highly {{c1::reduced}} and {{c1::compact}} because they are stored…
Published	07/28/2024	Oxidation of unsaturated fatty acids requires {{c1::isomerase}} and {{c1::NADPH}} to reorganize double bond for oxidation
Published	07/28/2024	There are many genetic deficiencies that cause fatty acid metabolism disorders that generally do not cause complete {{c1::loss}} of function, and can …
Published	07/28/2024	Glucose {{c1::cannot}} be made from Acetyl-CoA because pyruvate dehydrogenase which converts pyruvate into acetyl coa is {{c1::irreversible}}
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