Proteins Flashcards
Linear polymers of amino acid
Proteins
The set of all the proteins
Proteome
Identify the entire complement of proteins
Proteomics
Each amino acid has
Carboxyl group
Amino group
R-group
R-group dictates
The function of the amino acid in a protein
Amino acid: Has the smallest side chain
Glycine
Used in the first step of heme synthesis
Glycine
Major inhibitory neurotransmitter in the spinal cord
Glycine
Carrier of ammonia and of the carbons of pyruvate from skeletal muscle to liver
Alanine
Branched-chain amino acids
Valine
Leucine
Isoleucine
Precursor of tyrosine
Phenylalanine
Amino acid: Has the largest side chain
Tryptophan
Precursor of homocysteine
Methionine
Not an amino, but an imino acid
Proline
Amino acids: Contain a polar hydroxyl group
Serine
Threonine
Tyrosine
Precursor for thyroxine and melanin
Tyrosine
Amino acids: Have a carbonyl group and an amide group
Asparagine
Glutamine
Amino acid: Site for N-linked glycosylation of proteins
Asparagine
Major carrier of nitrogen to the liver from peripheral tissues
Glutamine
Amino acid: Contains a sulfhydryl group
Cysteine
Amino acid that participate in the biosynthesis of coenzyme A
Cysteine
Two cysteines can be connected by a covalent disulfide bond to form
Cysteine
Serve as proton donors
Aspartate
Glutamate
Precursor for GABA and glutathione
Glutamate
Proton acceptors
Histidine, Arginine, Lysine
Precursor of histamine
Histidine
Also used in the diagnosis of folic acid deficency
Histidine
Precursor of creatinine, urea, and nitric oxide
Arginine
21st amino acid
Selenocysteine
Amino acid: Considered nutritionally semiessential
Arginine
Amino acids: Synthesized in the body, but only from essential amino acid precursors
Cystine
Tyrosine
Determined by a proteins amino acid sequence
Primary Structure
Fatal neurodegenerative disease resulting from deposition of insoluble protein aggregates in neural cells
Prion disease
Prion disease in human
Creutzfeld Jakob disease
Prion disease in sheep
Scrapie
Prion disease in cattle
Bovine spongiform encephalopathy
Prion disease in cannibalistic tribes
Kuru
Most common and most important degenerative disease of the brain.
Alzheimer Disease
Heme protein found exclusively in
Red blood cells
Hemoglobin exists in 2 configurations
Taut form - low oxygen affinity
Relaxed form - high oxygen affinity
Following massive crush injury, _______ released from damaged muscle fibers colors the urine dark red.
Myoglobin
Myoglobin is found in
Heart, Skeletal Muscle
Cut off of HbA1c
More than or equal to 6.5 %
For microvascular disease prevention, the A1C goal is
<7 %
Form of hemoglobin bound to carbon monoxide in place of O2
Carboxyhemoglobin
Hb becomes cherry pink in color
Carboxyhemoglobin
Treatment for Carboxyhemoglobin
100 % O2 theraphy
Oxidized form of hemoglobin (Fe3+) that does not bind O2
Increased affinity for cyanide
Methemoglobin
Symptoms of Methemoglobinemia
Anxiety
Headache
Dyspnea
Chocolate cyanosis and O2 saturation is at 85 %
Methemoglobin
Treatment for Methemoglobin
Mild
Massive
Mild: Oral Methylene blue or Ascorbic Acid
Massiv: IV Methylene blue
Erythrocytes spheroidal, less deformable, vulnerable to splenic sequestration and destruction
Hereditary Spherocytosis
Most common mutation in Hereditary Spherocytosis
Ankyrin
Clinical Manifestation of Hereditary Spherocytosis
Anemia
Splenomegaly
Jaundice
Diagnostic test for Hereditary Spherocytosis
Osmotic Fragility Test
Results from a point mutation in both genes coding for the B-chain that results in a VALINE rather than a glutamate
Sickle Cell Disease
Clinical manifestation of Sickle Cell Disease
Anemia
Tissue anoxia
Painful crises
PROTECTIVE AGAINST MALARIA
Hemoglobin variant that has a single amino acid substitution in the 6th position of the B-globin chain, in which LYSINE is substituted for glutamate
Hemoglobin C Disease
Inadequate synthesis of a-chains
Alpha Thalassemia
Alpha Thalassemia leads to
Anemia
Accumulation of Hb Bart and Hb H
B-chain precipitation
Inadequate synthesis of B-chains
A-chain precipitation
Appear only after Birth
Beta Thalassemia
Most abundant protein in the body
Collagen
Collagen is stabilized by what bond?
Hydrogen bonds
Most common form of collagen
Type 1 collagen
Collagen is rich in
Glycine
Proline
Collagen monomers aggregate and become cross linked to form
Collagen fibrils
Hyperextensibility of the skin
Abnormal tissue fragility
Increased joint mobility
Ehlers-Danlos Syndrome
Britte bone syndrome
Osteogenesis Imperfecta
Mutation of genes in Osteogenesis Imperfecta? Marfan Syndrome?
Osteogenesis Imperfecta - Collagen
Marfan Syndrome - Fibrillin
Genetic disorders affecting the type IV collagen fibers
Alport Syndrome
Main presenting sign of Alport Syndrome
Hematuria
The skin breaks and blisters
Defect in type VII collagen
Epidermolysis Bullosa
Characterized by kinky hair and growth retardation
Menkes Disease
Connective tissue protein with rubber like proteins
Elastin
Elastin is rich in
Proline and Lysine
Mutation in the fibrillin gene
Marfan Syndrome
If there is DECREASED A-1 ANTITRYPSIN in the lungs, elastase destroys the alveolar walls, resulting to
EMPHYSEMA