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Protein Metabolism I (Catabolism (Transamination (Non Essential Amino…
Protein Metabolism I
Catabolism
To form ATP
Amino Acid Carbon Skeletons
Alpha ketoglutarate
Succinyl-CoA
Fumarate
Oxaloacetate
Pyruvate
Glucogenic (to form glucose)
AA Carbon Skeletons EXCEPT LEUCINE AND LYSINE
Alpha ketoglutarate
Succinyl CoA
Fumarate
Oxaloacetate
Pyruvate
Transamination
Components
Products: alpha keto acid and glutamate
Substrates: amino acid + alpha ketoglutarate
Non Essential Amino Acids
Oxaloacetate
Aspartate
Asparagine
Alpha ketoglutarate
Proline
Arginine
Glutamate
Glutamine
Pyruvate
Alanine
Essential Non Essentials
Serine+ Methionine
Cysteine
Phenylalanine
Tyrosine
Ketogenic (to form ketone bodies)
Amino Acids: W, F, Y, T, I and LEUCINE AND LYSINE
Acetyl-CoA
Acetoacetyl CoA
Urea Cycle
Glutamine/Glutamate Shuttle
Attach Ammonium to glutamate in outside tissues to transfer as glutamine to liver. Then ammonium dissociates in the liver
Formation of arginine to get rid of excess nitrogen
Limiting Step: Carbamoyl Phosphate Synthase
Consequences
Sources of Amino Acids
Digestion
Zymogens
Protein Turnover
Calpains
Via cytosol
Ubiquitin Proteosome
Cathepsin
Via lysosome
Autophagy
Biosynthesis
Maple Syrup Disease
Branched Chain Alpha Ketoacid Dehydrogenase Deficiency
Leucine
Isoleucine
Valine
Symptoms
Delayed myelenation
Burnt sugar smell in urine
Treatment
TPP (B1) to increase BCKA dehydrogenase (in some mutants)
Gelatin for dietary protein
Reduce intake of branched chain AA
Amino Acid Pool
Essential AA
Isoleuicine
Methionine
Tryptophan
Histidine
Threonine
Alanine
Valine
Leucine
Proline
Lysine
Conditional
Cysteine
Arginine
Tyrosine
Maintenance
Intake = Outtake (100g)
Synthesis=Breakdown (300 g)
Heme
Synthesis
Limiting step: Aminolevulenic acid Synthase (ALS)
Substrates: succinyl CoA + glycine
Disorders
Conjugated hyperbillirubinemia
Defect in ABC transporter
Kernicterus
Persistent unconjugated hyperbillirubinemia
Defect in UDP-Glucuronyltransferase
Drug Induced Hyperbillirubinemia
Failure to take up albumin
Transient jaundice
In neonates
Resolves after 72 hours
Due to increased hemolysis of fetal Hb
Porphyrias
Erythropoeitic
Leads to skin sensitivity/rash
Hepatic
Acute Intermittent
Defect in porphobilinogen deaminase