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 matrixlong-chain fatty acids not transferred to carnitinelong-chain fatty acids remain attached to carnitine in matrix

CPT I deficiency is when long-chain fatty acids remain attached to carnitine in the matrix because…

Answered: Deficiencies of carnitine, carnitine…

Biochemistry

9th Edition

ISBN:9781319114671

Author:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.

Publisher:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.

Chapter1: Biochemistry: An Evolving Science

Section: Chapter Questions

Problem 1P

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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.
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
describe the development of lactose intolerance from child age to adult age. also describe the detailed mechanism and molecular and enzymatic pathway related to development of lactose intolerance.
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
A lack of the enzyme glucose 6-phosphatase is one of the causes of the glycogen storage disorder von Gierke disease. Predict three signs or symptoms of von Gierke disease. hyperlipidemia (high lipid concentration in blood) normal glycogen structure, with very large amounts present in liver cirrhosis of the liver increased amounts of lactate and pyruvate in blood muscle cramps
Describe roles of each non-essential amino acid: ala, asp, asn, glu, gln, arg, gly, ser. Describe roles of each essential amino acid: cys, met, phe, tyr, try, and BCAAs. Describe the importance of one carbon metabolism and homocysteine. Describe the best indicator(s) of protein status. What is the purpose of an SGA? Give examples of physical signs of protein deficiencies. Simple to the point answers please!
Identify if True or False The activated form of glucose that serves as glucose donor for glycogen synthesis is formed by the reaction of UTP and glucose 1-phosphate. True False
Write the name of different types of glycogen storage disorder and their enzyme deficiency.
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. release of glucagon in the blood to the activity of carnitine acyl transferase 1 2. phosphorylation of acetyl CoA carboxylase 3. low [carbon dioxide]/[oxygen gas] ratio to the oxygenase activity of RuBisCO
Discuss the regulation of fatty acid metabolism by the isozymic forms of acetyl CoA carboxylase. Explain the reciprocal control of fatty acid synthesis and degradation and the various means by which this control is achieved

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