Clinical Chemistry Exam 1 Lecture 3 – Flashcards
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Amino Acids are the building blocks of ... |
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proteins and nonprotein nitrogen compounds |
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What do Amino Acids Regulate? |
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Growth Repair Maintenance of all cells |
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Structure of Amino Acid |
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Amino Group (NH2) Carboxyl Group (COOH) Side Chain (R) |
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Source of Amino Acids: ____ synthesized in sufficient quantities ____ "essential" |
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11 9 |
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Where must the 9 essential AAs be supplied from? |
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Dietary proteins |
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Body Pool of Amino Acids |
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Diet Newly synthesized Those released from protein breakdown |
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Aminoacidopathies |
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Rare, inherited "inborn errors" of amino acid metabolism |
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Phenylketonuria (PKU) |
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Phenylalanine accumulates |
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What illnesses are associated with PKU? |
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Mental disabilities Seizures Behavioral Problems |
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What should you treat PKU with? |
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Restrictive Diet |
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What is the statistics with PKU? |
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1:10,000-15,000 |
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What odor in the urine is associated with PKU? |
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Musty |
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Does Arkansas require PKU to be screen in newborns? |
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Yes |
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Tyrosinemia |
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3 types ranging in mild to severe Temporary in some preemies |
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What is the statistics on Tyrosinemia? |
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1:100,000 |
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What are the 5 illnesses associated with Tyrosinemia? |
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Failure to thrive, mental defects, vision problems, liver & kidney failure |
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Alkaptonuria |
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Homogentistic acid Urine darkens upon exposure to air |
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What are the 4 illnesses associated with Alkaptonuria? |
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Arthritis Heart Problems Kidney stones in mid-30s Skin pigmentation |
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What is the statistics on Alkaptonuria? |
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1:250,000 |
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Maple Syrup Urine Disease |
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Sweet odor or urine and breath |
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What are the 3 substances associated with Maple Syrup Urine Disease? |
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Leucine Isoleucine Valine |
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What are the 4 illnesses associated with MSUD |
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Failure to thrive, seizures, coma, death |
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What is the statistics on MSUD? |
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1:185,000 |
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What are the 3 illnesses associated with Homocystinuria |
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Nearsightedness Abnormal blood clotting Brittle Bones |
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What are the 2 less common illnesses associated with Homocystinuria? |
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Mental defects Abnormal growth |
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What is the Statistics on Homocystinuria? |
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1:200,000 |
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What is the statistics on Cystinuria? |
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1:10,000 |
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What are the 3 illnesses associated with Cystinuria? |
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Defect in reabsorption/transport system Kidney stones at a young age Recurring UTIs |
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Albinism |
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4 types. Lack of melanin in skin, hair, and eyes. Photosensitivity and astigmatism. |
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What are people with Albinism at an increased risk for? |
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Melanoma |
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What is the statistic on Albinism? |
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1:20,000 |
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Proteins are found in all ____, ____, ____, and _____ |
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Cells Fluids Secretions Excretions |
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What is the composition of proteins? |
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16% Nitrogen Carbon Oxygen Hydrogen Sulfur |
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Linked amino acids create what kind of bond? |
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Peptide |
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Most average 200-300 amino acids to form a ______ ______ |
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polypeptide chain |
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Primary Structure of Proteins |
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The specific sequence of amino acids; |
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Secondary Structure of Proteins |
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Chains coil creating pleated sheets or helix |
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Tertiary Structure of Proteins |
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When helix folds to create 3 dimensional structure |
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Quaternary Structure of Proteins |
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When 2 or more polypeptide chains join to form a larger protein |
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What does the Primary structure of Proteins determine? |
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The function of the protein |
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What is an example of a Quaternary structure? |
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Hemoglobin |
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Conjugated Protein |
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Protein + Nonprotein group |
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Conjugated Protein: Lipid + Protein = Heme + Globin = |
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Lipoprotein Hemoglobin |
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True or False: Proteins are constantly synthesized and degraded |
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True |
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Metabolism/Catabolism of Proteins is often associated with which organs? |
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Liver Lymph nodes Spleen |
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Nitrogen Balance = |
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Intake/Synthesis - Degradation/excretion |
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Disintegration of proteins occurs where? |
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GI tract Kidneys Liver |
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Proteins break down to amino acids which can then be ___, ____, or _______ (____ ___ ) |
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Recycled Re-used Re-purposed (Kreb's Cycle) |
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The products of Amino Acids being broken down are? |
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Ammonia Energy CO2 H20 |
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Liver converts ammonia into ______ which is excreted into urine |
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urea |
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All of the following are considered what? Hormone receptors pH buffers Water balance Osmotic pressure Transporters of small molecules Energy and nutritional source for tissues (12-20%) Structural role (collagen) Enzyme catalysts Protective role (immunoglobulins) Clotting factors Muscle contractions |
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Functions of Proteins |
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What will cause decreased protein levels? |
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Burns, wounds, blood loss Malnutrition or starvation Liver disease Renal disease Malabsorption/GI Disease |
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What will cause increased protein levels? |
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Dehydration Chronic inflammation or infection Hemolysis (false +) |
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What 2 diseases are associated with increased protein levels? |
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MM & Waldenstrom's |
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Denaturation |
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When the structure is disturbed, the protein's function is lost |
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What causes Denaturation? |
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Heat Repeated freeze-thaw cycles pH extremes UV light Chemicals, detergents |
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What can cause a falsely elevated protein level? |
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Hemolysis |
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Where are most proteins synthesized? |
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Liver |
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Where are the immunoglobulins synthesized? |
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Lymph Nodes/Spleen |