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20 Cards in this Set

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