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Factors that contribute to nitrogen balance
AA pool:
- Protein Degradation: (1) ATP-dependent ubiquitin proteasome that degrades damaged or short-lived proteins (2) ATP-independent system in lysosomes that degrades intra and extracellular proteins
- Diet
- Endogenous Synthesis of Nonessential AAs
AA consumption:
- Synthesis of proteins and other nitrogen containing compounds
- Conversion of AAs to glucose, glycogen, FAs and/or ketones
Amino Acid Catabolism
1. Transfer of the alpha amino group from the AA to
alpha-ketoglutarate to form an alpha-keto acid and glutamate (or aspartate)
2. Glutamate undergoes deoxygenation to form free ammonium and
alpha-ketoglutarate by glutamate dehydrogenase
3. Ammonium and aspartate are metabolized to urea via urea cycle
Alanine Aminotransferase (ALT)
Transfers AA group from alanine to alpha-ketoglutarate to form pyruvate and glutamate
Aspartate Aminotransferase (AST)
Transfers AA group from glutamate to OAA to form aspartate and alpha-ketoglutarate
Purpose of urea cycle
Convert insoluble toxin ammonium into a water soluble form (urea) that can be excreted in the urine
Starting products and end products of urea cycle
Starting: ammonium and HCO3
Ending: urea and ornithine
OTC Deficiency
Ornithine Transcarbamylase Deficiency
- X linked inheritance
- Hyperammonium, elevated glutamine, low citrulline
- "Intoxication" - vomiting, AMS, coma, respiratory alkalosis
- Acute Treatment: discontinue protein, administer dextrose and lipids
- Chronic Treatment: low protein diet, supplemental AAs, oral sodium phenylbutyrate, liver transplant
PA/MMA
Propionic Acidemia and Methylmalonic Acidemia
- Autosomal Recessive
-Propinoyl-CoA carboxylase or Methylmalonyl-CoA mutase deficiency
-VOMIT (valine, odd chain FAs, methionine, isoleucine, threonine) catabolism
-acidosis, ketosis, ketonuria, elevated VMIT, elevated methylmalonic acid and methylcitrate
- Intoxication - vomiting, AMS, coma
- Acute treatment: discontinue protein, dextrose, lipids, fluids, carnitine
- Chronic treatment: restrict VOMIT intake, supplement other AAs, carnitine, liver and/or kidney transplant
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