Untitled Quiz

Study Notes

Takes place in the mitochondrial matrix.
Acts as a central hub for metabolizing carbohydrates, fats, and proteins.
Oxidizes acetyl groups from acetyl-CoA to CO2, producing NADH and FADH2.
Begins with acetyl-CoA combining with oxaloacetate to form citrate and ends with the regeneration of oxaloacetate.
Key enzymes include citrate synthase, aconitase (requires Fe2+), isocitrate dehydrogenase (requires NAD+), and others.
Produces 10 ATP per acetyl-CoA via 3 NADH, 1 FADH2, and 1 GTP, with contributions from the respiratory chain.
Regulation involves allosteric inhibition by ATP and NADH, and activation by ADP and NAD+.

Refers to glycogen synthesis from glucose.
Primarily occurs in the liver and skeletal muscle.
Involves converting glucose to glucose-6-phosphate, then to glucose-1-phosphate, and finally to UDP-glucose before being added to glycogen.
Branching is facilitated by branching enzyme, creating α-1,6 glycosidic bonds.
Glycogenin acts as a primer for starting glycogen synthesis.

Defines the breakdown of glycogen into glucose-1-phosphate.
Occurs in the liver for blood sugar regulation and in muscle for local energy needs.
Key enzymes include glycogen phosphorylase (cleaves α-1,4 bonds) and debranching enzyme (handles α-1,6 branch points).
Process begins with glycogen and Pi producing glucose-1-phosphate and glycogen(n-1).
In the liver, glucose-6-phosphate is converted to glucose by glucose-6-phosphatase.
Regulation activated by glucagon and epinephrine, and inhibited by insulin.

Von Gierke's Disease (Type I): Caused by glucose-6-phosphatase deficiency, leading to glycogen accumulation in liver and kidney.
Pompe's Disease (Type II): Result of lysosomal α-1,4-glucosidase deficiency, causing glycogen to accumulate in lysosomes.
Cori's Disease (Type III): Linked to debranching enzyme deficiency, resulting in accumulation of limit dextrin.
Andersen's Disease (Type IV): Deficiency in branching enzyme, leading to abnormal glycogen with few branches.
McArdle's Syndrome (Type V): Caused by muscle phosphorylase deficiency, resulting in reduced exercise tolerance.

Functions to produce NADPH and ribose-5-phosphate.
Located in the cytosol of the liver, adipose tissue, adrenal cortex, and red blood cells.
Key steps include the formation of 6-phosphogluconolactone and ribulose-5-phosphate, generating NADPH and CO2.
Involves non-oxidative interconversions of sugars with varying carbon counts.

Involves synthesizing glucose from non-carbohydrate sources.
Primarily occurs in the liver and kidney.
Key substrates are lactate, amino acids, and glycerol.
Important enzymes include pyruvate carboxylase, phosphoenolpyruvate carboxykinase, fructose-1,6-bisphosphatase, and glucose-6-phosphatase.
Regulation is stimulated by glucagon and cortisol, and inhibited by insulin.