Amino Acid Metabolism PDF

Molecular Fundamentals of Medicine Amino Acid Metabolism 1/7/25 BMB Chapter 37 1 1  Amino Acid Metabolism 1. Essential AA’s & Nitrogen Balance 2. TCA Cycle: Glycogenic vs. Ketogenic 3. Cystinuria 4. Maple Syrup Urine Disease 5. P...

Molecular Fundamentals of Medicine Amino Acid Metabolism 1/7/25 BMB Chapter 37 1 1  Amino Acid Metabolism 1. Essential AA’s & Nitrogen Balance 2. TCA Cycle: Glycogenic vs. Ketogenic 3. Cystinuria 4. Maple Syrup Urine Disease 5. PKU 6. Alkaptonuria 7. Homocystinuria 1/7/25 2 2  Amino Acid Biosynthesis 1/7/25 3 3  Nitrogen Balance Not all amino acids can be synthesized by the human body. An essential amino acid deficiency leads to Negative Nitrogen Balance; whereby, nitrogen excretion exceeds intake. Positive Nitrogen Balance: nitrogen intake exceeds excretion. 1/7/25 4 4 1/7/25 5 5  TCA Cycle Intermediates 1/7/25 6 6  Glycogenic (Glucogenic) & Ketogenic 1/7/25 7 7  Ketogenic 1/7/25 8 8 1/7/25 9 9  Disorders of Amino Acid Metabolism 1/7/25 10 10  Cystinuria 1/7/25 11 11 1/7/25 12 12 Cystine 1/7/25 13 13  Homocyst(e)ine 1/7/25 14 14  Branched Chain Amino Acids 1/7/25 15 15  Maple Syrup Urine Disease Urine has an odor of maple syrup Mental retardation Abnormal muscle tone Ketosis Coma Death 1/7/25 16 16  Phenylalanine & Tyrosine Metabolism 1/7/25 17 17  Phenylalanine Hydroxylase © Dihydropterdine Reductase 1/7/25 18 18  Phenylketonuria, PKU Mental retardation Microcephaly Diet low in Phe Avoid aspartame Diet important during pregnancy Musty odor 1/7/25 19 19 Homogentisic Acid Oxidase 1/7/25 20 20  Triad of Alkaptonuria 1. Dark Urine 2. Ochronosis: Darkening of the joints. 3. Arthritis 1/7/25 21 21  Propionic Acid Pathway of Valine Or Methionine Isoleucine Threonine Is VOMIT 1/7/25 22 22  1) Cystathionine Synthase 2) Cystathionine Lyase 3) Homocysteine Methyltransferase Homocystinuria Fig. 39.12 1/7/25 23 23 Homocystinuria Fig. 39.6 Dislocated lens Deep vein thrombosis Stroke Atherosclerosis Mental retardation 1/7/25 24 24 Methylmalonic Acidurea: diagnostic for a Vitamin B12 deficiency 1/7/25 25 25  1988-2025 1969-1972 1/7/25 26 26 1/7/25 27 San Francisco Bay departing for WestPac (S. China Sea), November 1971 27 1/7/25 28 USS Coral Sea CVA43 in Pearl Harbor 23-26 November 1971 28 1/7/25 29 Thanksgiving Day 1971 North Oahu Hawaii 29 1/7/25 30 USS Tappahannock AO43 at sea replenishment of Coral Sea CVA43 30 1/7/25 31 VF111, USS Coral Sea CVA43, WestPac ‘71-’72 31 1/7/25 32 AT2 D M Kiick USN NAS Cubi Pt RP 1972 32 1/7/25 33 VF111 (F4 Phantom) “Sundowners” USS Coral Sea CVA43 (Nov71-Jul72) 33