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Amino Acid Metabolism
Single amino acids circulating in blood
Cellular Uptake
Used as precursors for N-containing compounds
Carbon used as sources of energy
Nitrogen must be removed in order to be excreted
Cellular uptake of amino acids: Specific transporters
Differ in:
Specificity
Expression
Defects in:
Cystinuria
Hartnup Disease
Glucogenic Degradation of Amino Acids
Carbons used to create glucose in the liver
- Produce intermediates of the TCA Cycle
All non-essential amino acids can be used to produce glucose
Ketogenic Degradation of amino acids
Carbons used to create ketone bodies or their precursors
- Acetyl-CoA or acetoacetate
Lysine and leucine are strictly ketogenic
amino acids that can be glucogenic or ketogenic
Tryptophan, isoleucine, threonine, phenylalanine and tyrosine
Nitrogen Balance
When balanced, the amount of nitrogen incorporated into macromolecules each day exactly equals the amount excreted from the body.
Positive nitrogen balance occurs during growth
- Amount incorporated exceeds the amount excreted
Negative nitrogen balance occurs from malnutrition
- Amount excreted exceeds the amount incorporated
Marasmus
Reduction in the total number of calories in an infant’s diet
child’s weight falls below 60% for normal sex, height and age
significant loss of body fat and muscle occurs due to catabolism of fats and protein, an adaptive strategy to use lipids and amino acids as energy sources.
Kwashiorkor
A protein-energy malnutrition (PEM)
Protein deprivation with adequate caloric intake
normal or nearly normal body weight and height for age
Often, the child has been weaned too early and subsequently fed a low-protein, high-carbohydrate diet.
Essential Amino Acids and Malnutrition
Insufficient essential amino acid intake results in decreased protein synthesis.
Hypoalbuminemia
- Albumin makes up 55-60% of the total serum protein.
- Albumin plays important roles in buffering and maintaining osmotic pressure.
- – Results in generalized edema and distended abdomen
Hypopigmentation
essential amino acid for kids but not adults
arginine
Synthesis of Amino Acids
Non-Essential Amino Acids:
9 synthesized from glucose alone
Tyrosine synthesized from phenylalanine
Cysteine acquires sulfur from methionine (essential)
Review: Maintenance of Blood Glucose Levels
Maintained by liver
Glycogenolysis
During fasting
Mostly depleted after more than 12 hours
Gluconeogenesis
During starvation
Increases as glycogen reserves decrease
Only source after 24 hours of fasting
Glycogen
Mainly in liver and skeletal muscle
a-1,4-glycosidic bonds
-Make up glycosyl units
a-1,6- glycosidic bonds
- Initiate branching
Glycogen Formation Process
- Glucose is phosphorylated to G-6-P
- Formation of UDP-Glucose
- Glycogen synthase
- – Transfers glucose units from UDP-G to the end of glycogen chains
- Branching enzyme
- – Moves units to form a branch
Glycogenolysis
Glycogen phophorylase
- Removes glucose units one at a time from ends forming G-1-P
Debranching enzyme
- Removes a-1,6 linked glucose residues as G-1-P
Phosphoglucomutase
- Creates G6P from G1P
Glucose-6-phosphatase
- Only in liver
- Converts to glucose to enter bloodstream