1. Essential and Non-essential amino acids Kwashiorkor marasmus Standard and Non-standard amino acids Nitrogen balance Positive:children,pregnancy and after …. Negative:aging,Fever and Cachexia

2. Cachexia wasting syndrome The formal definition is the loss of body mass that cannot be reversed nutritionally loss of weight muscle atrophy fatigue weakness significant loss of appetite cancer, AIDS, celiac disease, chronic obstructive pulmonary disease, multiple sclerosis, congestive heart failure, tuberculosis, familial amyloid polyneuropathy, mercury poisoning

3. Protein and amino acids Gasterin: HCl and pepsinogen Exo and endopeptidase Amino and carboxy peptidase Secretin(HCO3) and cholecystokinin (Bile and Enz) Tripsinogen(Entropeptidase) Chymotripsinogen Proelastase Procarboxy peptidase(Zn)

4. Serin protease SerPin DIFP(Cholin esterase inhibitor) SAT by Na : AA, Di and Tripeptide Na for amylase activity

5. αKG and Glu

6. ALT or GPT AST or GOT PLP as a cofactor(ε amin of Lys) Isoniazid deritis ratio: acute viral hepatitis : ALT was usually higher than AST alcoholic hepatitis:AST is usually higher than ALT

7. acute viral hepatitis can have AST greater than ALT, and this can be a sign of fulminant disease alcoholic hepatitis can have ALT greater than AST when several days have elapsed since alcohol exposure short half-life of AST (18 h) compared to ALT (36 h) chronic viral hepatitis and chronic alcoholism as well as non-alcoholic fatty liver disease, an elevated AST/ALT ratio is predictive of long terms complications including fibrosis and cirrhosis

8. Transamination Heart of aa metabolism Thr and Lys α amin transamination or α carbon:PLP

9. Glutamate dehydrogenase GDH Mitochondrial GTP(-) and ADP(+) Reverse reaction in prevenous Intestinal bacteriae Nitrogen fixation Oxidative deamination

11. Nitrogen fixation

22. Urea cycle disorders Hyperammonemia encephalopathy respiratory alkalosis most severe when the metabolic block occurs at reactions 1 or 2 Clinical symptoms include vomiting, avoidance of high-protein foods, intermittent ataxia, irritability, lethargy, and severe mental retardation The clinical features and treatment of all five disorders are similar

23. Carbamoyl Phosphate Synthase I hyperammonemia type 1 Fatal in babies Late strikes in childhood Seizure,Nausea and Abdominal pain N-Acetylglutamate Synthase N-Acetylglutamate is essential for the activity of carbamoyl phosphate synthase I indistinguishable from those arising from a defect in carbamoyl phosphate synthase I a deficiency in NAGS may respond to administered N- acetylglutamate.

24. Ornithine Permease Hyperornithinemia hyperammonemia homocitrullinuria syndrome (HHH syndrome) ORNT1 (mitochondrial ornithine transporter 1) Ornithine Transcarbamoylase "hyperammonemia type 2“ Elevated level of amino acids, hyperammonemia and Orotic acid X-chromosome linked More sever in heterozygote male than that of female glutamine are elevated in blood, CSF, and urine

25. Argininosuccinate Synthase Citrullinemia a 25-fold elevated Km for citrulline has been reported Citrullinemia CSF citrulline level are elevated 1–2 g of citrulline are excreted daily

26. Argininosuccinate Lyase Argininosuccinicaciduria argininosuccinate in blood, CSF, and urine friable, tufted hair (trichorrhexis nodosa) Death to two years Diagnosis by the measurement of erythrocyte argininosuccinate lyase activity can be performed on umbilical cord blood or amniotic fluid cells.

27. Arginase Hyperargininemia: Blood,CSF and urin the first symptoms of hyperargininemia typically do not appear until age 2 to 4 years. The urinary amino acid pattern resembles that of lysine-cystinuria may reflect competition by arginine with lysine and cysteine for reabsorption in the renal tubule.

28. Non-protein nitrogen compounds NPNs Urea: most abundant compound Urea:15-45 mg/dl (European countries) BUN: 7-20 mg/dl (USA) Uric acid Creatinine

29. Treatment Decreasing intake of proteins Intake of α-ketoacids Intake of lactulose