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8 Cards in this Set
- Front
- Back
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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 |
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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 |
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Alanine Aminotransferase (ALT) |
Transfers AA group from alanine to alpha-ketoglutarate to form pyruvate and glutamate |
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Aspartate Aminotransferase (AST) |
Transfers AA group from glutamate to OAA to form aspartate and alpha-ketoglutarate |
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Purpose of urea cycle |
Convert insoluble toxin ammonium into a water soluble form (urea) that can be excreted in the urine |
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Starting products and end products of urea cycle |
Starting: ammonium and HCO3 Ending: urea and ornithine |
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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 |
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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 |
