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Clinical Chemistry Exam 1 Lecture 3
Amino Acids and Proteins
59
Chemistry
Undergraduate 3
01/31/2015

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