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Mapot Sapling Learning Deficiencies of carnitine, carnitine acyltransferases, or

ID: 585308 • Letter: M

Question

Mapot Sapling Learning 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. Šymptom severity varies with the form of the deficiency The following abbreviations are used: carnitine acyltransferase (carnitine palmitoyltransferase), CPT and carnitine/acylcarnitine translocase, CACT ldentify each symptom or effect as a deficiency of CPT I, CPT II, CACT, or both CPT I and CACT. One bin will remain empty CPT I deficiency CPT II deficiency CACT deficiency CPT I or CACT deficiency long-chain fatty acids remain attached to carnitine in matrix acyl carnitine not transported across inner mitochondrial membrane carnitine not converted to acyl carnitine in cytoplasm You have misplaced the phrase that describes CPTI Recall that CPT I transfers an acyl group from coenzyme A to the hydroxyl group of carnitine. What product is formed?

Explanation / Answer

Matching each symptom or effect as a deficiency of CPT I, CPT II, CACT, or both CPT I and CACT

CPT deficiency

Long chain fatty acids remain attached to carnitinein matrix

CPT II deficiency

deficiency

Empty box

CACT deficiency

acyl carnitine not transported accross inner mitochondrial membrane

CPT I or CACT deficiency

carnitine not converted to acyl carnitine in cytoplasm