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 |