Deficiencies of carnitine, carnitine acyltransferases, or carnitine/acylcarnitine translocase affect the metabolism of long-chainfatty acids. Many of the symptoms are similar, and include hypoketotic hypoglycemia, low ketone levels and low blood sugarwith fasting, and damage to the liver, heart, or muscles due to fatty acid buildup in those tissues. Symptom severity varies withthe form of the deficiency.The following abbreviations are used: carnitine acyltransferase (carnitine palmitoyltransferase), CPT and carnitine/acylcarnitinetranslocase, CACT.Identify each symptom or effect as a deficiency of CPT I, CPT II, or CACT.CPT I deficiencyCPT II deficiencyCACT deficiencyAnswer Bankacyl carnitine not transported into mitochondrial matrixacyl carnitine not converted to carnitine in matrixlong-chain fatty acids not transferred to carnitine

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Answered: Deficiencies of carnitine, carnitine…

Biochemistry

6th Edition

ISBN:9781305577206

Author:Reginald H. Garrett, Charles M. Grisham

Publisher:Reginald H. Garrett, Charles M. Grisham

Chapter22: Gluconeogenesis, Glycogen Metabolism, And The Pentose Phosphate Pathway

Section: Chapter Questions

Problem 8P

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In healthy adults, the concentration of glucose in blood is approximately80 to 110 milligrams per deciliter (mg/dl). After a carbohydrate-richmeal, however, the concentration may spike to 140 mg/dl. Describe thehormonal action that returns blood glucose to normal.
Cori’s disease, is an autosomal recessive metabolic disorder that is characterized by defects in the glycogen debranching enzyme amylo-1,6-glucosidase,4-α-glucanotransferase (AGL). It primarily effects the Liver, heart and skeletal muscle. Symptoms can include hyperlipidemia, ketoacidosis, and hypoglycemia. The possible dietary recommendations are as follows:1. A high protein diet to facilitate gluconeogenesis2. A ketogenic diet to provide an alternate source of energy3. Small frequent meals to prevent hypoglycemia4. A high fat dietWhich dietary recommendations can be used to treat this disorder? a) 2 and 4 b) 1 and 2 c) 2 and 3 d) 1 and 3 e) None of the above recommendations would be effective f) 3 and 4 g) 1 and 4
In 3-4 sentences, briefly explain how lactate is formed, the biological effect of lactate, and explain the biochemical process and path for conversion of lactate. 3. The hormones glucagon, epinephrine, and insulin, can regulate blood glucose levels to protect the brain. For each one provide a short explanation as to whether it raises or lowers glucose how it does this, and indicate whether it is metabolic or catabolic.
Describe five possible fates for glucose 6-phosphate in the liver.
Nonalcoholic fatty liver disease is thought to be exacerbated by a diet heavy in sugar. Explain?
Explain why the amino acid tryptophan is both ketogenicand glucogenic.
Describe the gluconeogenesis. What are the substrates for gluconeogenesis? (yellow boxes) and write the names of the enzymes belong to the gluconeogenic pathway. Glucose 4 se-6-phosphate usphoglucose isomerase Fructose-6-phosphate Fructose-1,6-bisphosphate HAidolase Triose phosphate Dihydroxyacetone phosphate isomerase Glyceraldehyde 3-phosphate P.. NAD CH,OH Glyceraldehyde 3-phosphate dehydrogenase 1,3-Bisphosphoglycerate CH,OPO,- NADH ADP Phosphoglycerate kinase ATP 3-Phosphoglycerate Phosphoglycerate || mutase 2-Phosphoglycerate 2 X Enolase Phosphoenolpyruvate GDP, CO, GIP Oxaloacetate ADP + P, 1 ATP, HCOS Pyruvate
B. Describe the function implied by the name of each of the following enzymes 1. Citrate decarboxylase 2. Adenine diphosphate phosphorylase 3. Oxalate reductase 4. Nitrite oxidase 5. cis-trans isomerase
Match the bold specific genetic metabolic disorders to their category: adrenal hyperplasia Albinism Cystinuria Galactosaemia Phenylketonuria Pompe Wilson Disease Chromosomal abnormality Excessive amounts of metabolites in a metabolic pathway Storage disease Disrupted regulatory system Disrupted Vitamin and/or co-factor absorption or processing Transport disorder Accumulation of substrates in a metabolic pathway Absence of end product
The glycolytic pathway is regulated by a number of key enzymes. Describe the various substrates and hormones that act on these enzymatic steps and the type of regulation (e.g. allosteric etc.).
Which glycogen storage disorder is known as amylopectinosis? explain very briefly.
Indicate what will happen (increase, decrease or no effect) to the activity of enzyme or rate of the metabolic pathway given the following conditions: 1. epinephrine to the activity of glycogen synthase 2. high [2-carboxyarabinitol-1-phosphate] to the carboxylase activity of RuBisCO

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