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20 Cards in this Set
- Front
- Back
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Trypsinogen --- pos + NH3
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Lys and Arg
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Pepsin - Chymotrypsin
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FYW aromatic
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Hartnup disease
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neutral Trp no energy
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Cystinuria - Homocysteinuria S-S
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in Cys, lys, arg and ornithine -- kidney disease - blood clot - Vit B 6-9-12 (PLP-folic-coba) - -- retarded
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liver test
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– Alkaline phosphatase - Alanine Transaminase (ALT) •Aspartate Transaminase (AST) leakage -- inflam
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Aginio-succinate Synthetase deficiency
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= elevated ammonia and Citurline -- retarded
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Carbamoyl Phosphate Synthetase Deficiency
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= Elevated ammonia - -- retarded
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Glutamate deHydrogenase
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remove NH3 from glutamate (become a-ketoacid) -- so NH3 + Asp --> Urea cycle
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Crea-tine turn to
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Creatine phosphate is found in muscles and used as molecule to quickly replenish ATP
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Crea-ti-nine
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non-enzymatic decomposition of creatine -- for elimination
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Essential amino acid: 1 small ring - 4 branch-ketone-pos - 2 aro - T - M --> 9 total
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Val - Ile - Leu - Lys - His - Phe - Trp - - Thr - Met
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Maple Syrup Urine Disease deCarb- deH
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NO a-ketodehydrogenase can't break 1 ring 2 branches (Val- Ile - Leu) B 1 2 3 5 --- B 6 --- acidic urine blood - retarded
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Methylmalonyluria
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B12 dependent conversion to succinyl-CoA - chelate Ca- kidney stone -- Acidic blood - retarded
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PKU
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NO tetra-H-biop-terin (THB) can't Hydroxylation of Phe to Tyr - strict diet - no melanin -- no neurotransmister ----> the only retarded melanin related (vs Tyr1-2-3-alcap)
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Tyrosinemia 2
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to much Tyr - too much neurotrans - black palm
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Tyrosinemia 1
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too much Tyr at the end cabbage like odor --
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Alcapton-urine
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too much Tyr in the middle - black urine (melanine) - arthritic joint pain
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One of the minor degradation products of glycine ________ that complexes with calcium and is very water insoluble
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Oxalate - kidney stone
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Ala (A) - Glu (Q) are NH3 carriers of
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Muscle and other non-hepatic cells NH2-C=O
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Albinism Tyr 3
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can't make melonin - white
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