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

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

Indicate the following where the tissue(s) for the metabolic pathway takes place: Glycogenesis Lipogenesis Glycogenolysis Lipolysis Gluconeogenesis

Describe five possible fates for glucose 6-phosphate in the liver.