PPT-Inborn Errors of Amino Acid Metabolism

Renal Block Biochemistry of Phenylketonuria PKU Maple Syrup Urine Disease MSUD Albinism Homocyteinuria Alkaptonuria 1 Lecture Dr Ahmed H Mujamammi Inborn Errors of aa Metabolism. What are amino acids?. Amino acids are the building blocks of proteins.. In the body, they exist as zwitterions.. Zwitterions can behave as both an acid or a base.. Today we will:. Study . the acid-base properties of amino acids, . 15 . Slides. AMINO ACID . CATABOLISM. fig 18-1. fate of amino nitrogen in mammals. fig 18-2. metabolism of amino nitrogen in liver. fig 18-2. nitrogen removal molecules…. fig 18-2. metabolism of amino nitrogen in liver. 1 Usual presentation of inborn error of metabolismHealthy at birth .Decomposition occurring within days or weeks of birth. 2 Signs and Symptoms of Metabolic DiseaseFailure to thrive (weight below the Formation of Aminoacyl tRNAs. Ribosome structure. Stages of Translation. Relationship between DNA, mRNA, and Protein Sequences. Translation and the Genetic Code. Amino acid structure. Four different groups are attached to the central carbon atom (. We live in a human-centric world.. Life exists outside our box.. Subtitle. Text. Shock & Holland (2007). For example, there is life deep down on the ocean floor.. C-DEBI . (Center for Deep Energy Biosphere Investigations). Chapter 30, . Stryer. Short Course. Overview. Amino Acid Catabolism. Nitrogen removal. Urea Cycle. Metabolism of carbon backbone. Amino acid catabolism. Amino acids . from diet or protein turnover. Salvaged for use in proteins or catabolized. Stryer. Short Course, Chapter 31. Nitrogen fixation. Bacteria. Nitrogenase. Costly—16 ATP per N. 2. molecule. Assimilation into Amino Acids. In microorganisms/plants: assimilation of ammonia is key—synthesis of most amino acids. Nitrogen Metabolism. Nitrogen Forms in the Body. Nitrogen Balance Critical. Body Must Make and Break Down Amino Acids. Nitrogen Also Needed for Synthesis of. Nucleotides (ATP, GTP, CTP, UTP, dATP, dCTP, dGTP, dTTP). Richard D. Howells, PhD. Dental Biochemistry Lecture 25 . 2. Learning Objectives. To distinguish between phenylketonuria (PKU) caused by phenylalanine hydroxylase (PAH) defect and PKU caused by defect in . Richard D. Howells, PhD. Dental Biochemistry Lecture 24. 2. Learning Objectives. To describe the urea cycle and its fundamental role in the excretion of nitrogen.. To . distinguish between . glucogenic. Approximately 75% are reutilized.. The excess nitrogen forms urea.. Proteins represent 10-15 % of total energy supply.. Digestion and Absorption of Proteins.. The . α. -amino group of many amino acids is transferred to . Learning Objectives :. 1. Know the groups of . a.a. . biosynthetic families. 2. The enzymes and coenzymes involved in the synthetic pathways. 3. The enzyme deficiencies of each pathway.. 4. The consequences of the inborn errors of metabolism. GLYCINE. Glycine is the . simplest . amino acid. Chemically it is “. amino acetic acid. . ".. It is non-essential amino acid and can be synthesized in . tissues. . Though it is non- essential but it is an important amino acid as it forms many biologically important compounds in the body.. 1. Important. .. Extra Information.. Doctors slides. 436 Biochemistry team. One day or day one you decide ... By the end of this lecture the students will be able to:. • Identify the amino acid degradation and synthesis of non-essential amino acids..