1 / 16

MLAB 2401: Clinical Chemistry Keri Brophy -Martinez

MLAB 2401: Clinical Chemistry Keri Brophy -Martinez. Overview: Amino Acids & Proteins. Amino Acids. Building blocks of proteins Chemical properties determine biological activity Origin Majority made in the human Generated from amino acid pool Generated from breakdown of proteins

olympe
Télécharger la présentation

MLAB 2401: Clinical Chemistry Keri Brophy -Martinez

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. MLAB 2401: Clinical ChemistryKeri Brophy-Martinez Overview: Amino Acids & Proteins

  2. Amino Acids • Building blocks of proteins • Chemical properties determine biological activity • Origin • Majority made in the human • Generated from amino acid pool • Generated from breakdown of proteins • Essential Amino acids • Ingested in diet • “MILL PATH TV”

  3. Amino Acids • Linked together by peptide bonds to form the building blocks of proteins Peptide bond

  4. Aminoacidopathies • Rare inherited disorders • Enzyme defect that inhibits the body’s ability to metabolize certain amino acids • Abnormality due to problem with enzyme activity or the membrane transport system for amino acids • Cause severe medical problems due to the buildup of toxic amino acids and/or byproducts of amino acid metabolism in blood

  5. Aminoacidopathies: Disorders • Phenylketonuria • Absence of phenylalanine hydrolase • Mousy odor of the urine • Causes significant brain damage • Tyrosinemia • Three types • Type I most severe • Causes liver and kidney damage, affects CNS • Alkaptonuria • Absence of homogentisateoxidase • Urine turns brownish-black upon exposure to air

  6. Aminoacidopathies: Disorders • Maple syrup urine disease • Absence or reduction of alpha-ketoaciddecarboxylase • Hallmark feature is the odor of maple syrup or burnt sugar in the urine, breath and skin • IsovalericAcidemia • Absence of isovaleryl-CoAdehydrogenase • Distinctive odor of sweaty feet due to build up of isovaleric acid

  7. Aminoacidopathies: Disorders • Homocystinuria • Absence of cystathionine-beta-synthetase • Cystinuria • Defect in amino acid transport system • Results in the formation of stones

  8. Proteins • Built from one or more chains of amino acids • Specific • Contain carbon, hydrogen, oxygen, sulfur and nitrogen • Nucleotide sequence in the genes dictates the amino acid sequence of the protein

  9. Proteins • Synthesis • Plasma proteins made in liver then secreted by hepatocyte into circulation • Immunoglobulins made in plasma cells • Insufficient dietary quantities of amino acids will limit synthesis and lower body levels of proteins

  10. Proteins • Catabolism (Breakdown) • Goal is to remove nitrogen from the system • Occurs in the digestive tract, kidneys, and liver • Produces ammonia, then urea • Urea excreted via urine • Produces ketoacids • Ketoacids converted to glucose or fat

  11. Structure of Proteins

  12. Denaturation • Disruption of structure • Results in loss of function and protein chemical properties • Caused by: • Heat • pH changes • Mechanical forces • Exposure to UV light • Exposure to chemicals

  13. Classification of Proteins • Simple proteins • Proteins made of only amino acids • Further classified as globular or fibrous • Conjugated proteins • Proteins with nonprotein groups attached • Metalloproteins • Lipoproteins • Glycoproteins • Mucoproteins • Nucleoproteins • Phosphoproteins

  14. Functions of Proteins

  15. Functions of Proteins

  16. References • Bishop, M., Fody, E., & Schoeff, l. (2010). Clinical Chemistry: Techniques, principles, Correlations. Baltimore: Wolters Kluwer Lippincott Williams & Wilkins. • http://www.stanford.edu/group/pandegroup/folding/education/prstruc.html • Sunheimer, R., & Graves, L. (2010). Clinical Laboratory Chemistry. Upper Saddle River: Pearson .

More Related