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Nitrogen Balance,Excretion, Ornithine Transcarbamylase Def.-X-linked Dom -heterozygous FMs affected -most common gen. defect of urea cyc -Pt. lethargic, irritable, hypotonic ->convulsion, coma, death -Signs: hyperammonemia, alkalosis, low BUN, hyperglutaminemia, orotic aciduria -unique to OTC def. -other defs. w/ diff. accumulations Hyperammonemia Could be in premies w/ immature liver, low liver blood supply Prolonged->irrev. brain dmg, Nitrogen Balance Urea Cycle In Adults: Chronic Renal Failure->inc. BUN -min. protein intake~40g, with a-keto acid analogs of BCAAs Chronic Liver Dz->hepatic encephalopathy -dec. urea synth/catabolism of aromatic AAs ->hyperammonemia Protein intolerance-partial deficits in urea synth -avoid certain dietary pros. -present w/ hyperammonemic coma Lysinuric Protein intolerance -defective dibasic AA transporter ->K, R, ornithine in urine->can't support OTC -treat w/ oral citrulline, All Start as Zymogens, cleave peptide bonds at specific AAs: Stomach: -Pepsin(ogen)-self-activating by stomach H+ Pancreatic Enzymes:all activated by trypsin -Trypsin(ogen)-activated by enteropeptidase -EPase from brush border cells of sm. int. -Chymotrypsin(ogen) -(Pro)elastase -(Pro)carboxypeptidases A&B -remove AA at C terminus (R group specific) Intestinal Mucosa- aminopeptidases - intra- and extracellular -remove N-terminal AA Absorption of AAs Na+-dependent carriers into enterocyte -basolateral Na/K ATPase->cotransport ɳ semi-specific transport proteins -then leave ent'cyte via facil. xport. Hartnup Dz-int. cells can't abs. neutral AAs neither can renal cells->amino aciduria -aromatic or hydrophobic R groups: F,L,I,M,V,Y->Sx from deficiencies -other transporters are unaffected, Intermediate of pyrimidine synth -excess carbamoyl PO4->cytosol (from mito) ->lost of oratate via CPS-II CPS-I vs. CPS-II I-urea cyc, NH4-N source, mito,NAGA activateor II-nuc. synth,Gln-N source, cytosol, PRPP activator, Dietary & Endogenous Pros. Broken Down: -replace AAs->make proteins -ᡪ%visceral pros, 30%mm, 20% plasma -prev. AAs degraded to N cmpds -excess N -> urea Endogenous-digestive enz, mucosal lining Digestion of Proteins All Start as Zymogens, cleave peptide bonds at specific AAs: Stomach: -Pepsin(ogen)-self-activating by stomach H+ Pancreatic Enzymes:all activated by trypsin -Trypsin(ogen)-activated by enteropeptidase -EPase from brush border cells of sm. int. -Chymotrypsin(ogen) -(Pro)elastase -(Pro)carboxypeptidases A&B -remove AA at C terminus (R group specific) Intestinal Mucosa- aminopeptidases - intra- and extracellular -remove N-terminal AA, Na+-dependent carriers into enterocyte -basolateral Na/K ATPase->cotransport ɳ semi-specific transport proteins -then leave ent'cyte via facil. xport. Hartnup Dz-int. cells can't abs. neutral AAs neither can renal cells->amino aciduria -aromatic or hydrophobic R groups: F,L,I,M,V,Y->Sx from deficiencies -other transporters are unaffected AA Metabolism Aminotransferases->NH3 group transfer -from AA to acceptor alpha-ketoacid ->new AA and a-ketoacid (from old AA) -Asp+a-ketoglutarate<->Glu+OAA -via asp aminotransferase, AST -mostly in reverse, Glu, Asp->urea -Ala+a-ketoglutarate<->Glu+Pyruvate -via ala aminotransferase, ALT -pyruvate for GNG inc. ALT, AST indicate liver dmg, Nitrogen Balance Urea Cycle Ornithine Transcarbamylase Def.-X-linked Dom -heterozygous FMs affected -most common gen. defect of urea cyc -Pt. lethargic, irritable, hypotonic ->convulsion, coma, death -Signs: hyperammonemia, alkalosis, low BUN, hyperglutaminemia, orotic aciduria -unique to OTC def. -other defs. w/ diff. accumulations, Nitrogen Balance Proteins in and out Proteins Out- -most N out via UREA-made in liver Excess BUN->compromised renal fcn -major change if more N ingested -some also lost fecally, sweat, hair, etc. -higher protein intake->more urea excreted -fasting->mm breakdown for GNG, more urea, Sources of NH4 for urea cycle Bacterial N IN GUT, Nitrogen Balance Positive Nitrogen Balance Input>Output -growth, preg, lactation, recover from meta. stress -inc. prot synth, inc. input, NL output -MUST consume Add'l protein