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
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