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Deficiencies of carnitine, carnitine acyltransferases, or carnitine/acylcarnitine translocase affect the metabolism of long-chain fatty acids. Many of the symptoms are similar, and include hypoketotic hypoglycemia, low ketone levels and low blood sugar with fasting, and damage to the liver, heart, or muscles due to fatty acid buildup in those tissues. Symptom severity varies with the form of the deficiency. The following abbreviations are used: carnitine acyltransferase (carnitine palmitoyltransferase), CPT and carnitine/acylcarnitine translocase, CACT. Identify each symptom or effect as a deficiency of CPT I, CPT II, or CACT. CPT I deficiency CPT II deficiency CACT deficiency Answer Bank acyl carnitine not transported into mitochondrial matrix long-chain fatty acids not transferred to carnitine long-chain fatty acids remain attached to carnitine in matrix

Deficiencies of carnitine, carnitine acyltransferases, or carnitine/acylcarnitine translocase affect the metabolism of long-chain fatty acids. Many of the symptoms are similar, and include hypoketotic hypoglycemia, low ketone levels and low blood sugar with fasting, and damage to the liver, heart, or muscles due to fatty acid buildup in those tissues. Symptom severity varies with the form of the deficiency. The following abbreviations are used: carnitine acyltransferase (carnitine palmitoyltransferase), CPT and carnitine/acylcarnitine translocase, CACT. Identify each symptom or effect as a deficiency of CPT I, CPT II, or CACT. CPT I deficiency CPT II deficiency CACT deficiency Answer Bank acyl carnitine not transported into mitochondrial matrix long-chain fatty acids not transferred to carnitine long-chain fatty acids remain attached to carnitine in matrix

Biochemistry
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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Deficiencies of carnitine, carnitine acyltransferases, or carnitine/acylcarnitine translocase affect the metabolism of long-chain fatty acids. Many of the symptoms are similar, and include hypoketotic hypoglycemia, low ketone levels and low blood sugar with fasting, and damage to the liver, heart, or muscles due to fatty acid buildup in those tissues. Symptom severity varies with the form of the deficiency. The following abbreviations are used: carnitine acyltransferase (carnitine palmitoyltransferase), CPT and carnitine/acylcarnitine translocase, CACT. Identify each symptom or effect as a deficiency of CPT I, CPT II, or CACT. CPT I deficiency CPT II deficiency CACT deficiency Answer Bank acyl carnitine not transported into mitochondrial matrix long-chain fatty acids not transferred to carnitine long-chain fatty acids remain attached to carnitine in matrix
Transcribed Image Text:Deficiencies of carnitine, carnitine acyltransferases, or carnitine/acylcarnitine translocase affect the metabolism of long-chain fatty acids. Many of the symptoms are similar, and include hypoketotic hypoglycemia, low ketone levels and low blood sugar with fasting, and damage to the liver, heart, or muscles due to fatty acid buildup in those tissues. Symptom severity varies with the form of the deficiency. The following abbreviations are used: carnitine acyltransferase (carnitine palmitoyltransferase), CPT and carnitine/acylcarnitine translocase, CACT. Identify each symptom or effect as a deficiency of CPT I, CPT II, or CACT. CPT I deficiency CPT II deficiency CACT deficiency Answer Bank acyl carnitine not transported into mitochondrial matrix long-chain fatty acids not transferred to carnitine long-chain fatty acids remain attached to carnitine in matrix
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