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102 Cards in this Set

  • Front
  • Back
What is the difference between proteoglycans and glycoproteins?
Proteoglycans - Glycosaminoglycan chains (contains repeating disaccharides)

Glycoproteins - Oligosaccharide chains (no repeating disaccharides)
All glycosaminoglycans except hyaluronic acid are synthesized where?
Golgi

Hyaluronic acid synthesized in plasma membrane
Main locations of hyaluronic acid
cartilage, synovial fluid, vitreous humor of the eye
Medical uses of hyaluronic acid
Used in eye surgery and treatment of osteoarthritic pain
Most abundant glycosaminoglycan in the human body
Chondroitin sulfates
Increased accumulation of __________ in mitral valves is associated with thickening and abnormal displacement of the valve into the left atrium of the heart (mitral valve prolapse).
dermatan sulfate
The only glycosaminoglycan that does not contain an acidic sugar
Keratan sulfates
Glycosaminoglycan used as a anticoagulant in medicine
Heparin
The linkage region to which a GAG is attached to a protein to form a proteoglycan.
Hydroxyl of serine or amide of asparagine
Name for cartilage proteoglycan
Aggrecan
A group of lysosomal storage diseases.
Mucopolysaccharidoses
Functions of glycoproteins
Cell surface recognition (including binding sites for pathogens)
Blood group antigens
Extracellular matrix molecules
Mucins
Plasma proteins
Where is the oligosaccharide chain built during the synthesis of an O-linked glycoprotein?
Golgi
The only glucose transport protein that is insulin independent
GLUT-2
Glucose transport protein in muscle and adipose cells
GLUT-4
Glucose transport protein in hepatic cells
GLUT-2
Glucose transport protein in the brain
GLUT-1 and GLUT-3
Phosphorylation of glucose into G-6-P is catalyzed by:
Hexokinase
Enzyme the phosphorylates glucose in the liver. Has a high Km.
Glucokinase
The isomerisation of G-6-P into F-6-P is catalyzed by what enzyme?
Phosphoglucose isomerase
The phosphorylation of F-6-P to F-1,6-BP is catalyzed by what enzyme?
phosphofructokinase-1 (PFK-1)
Irreversible reaction in glycolysis. The "committed" step.
Phosphorylation of F6P
Enzyme that catalyzes the cleavage of F-1,6-BP
Aldolase
This compound inhibits enolase and results in a decrease in ATP synthesis
Fluoride
Inhibits glyceraldehyde 3-P dehydrogenase
Arsenate
Possible fates of pyruvate
Alanine (carrier of amino groups from muscle to liver)
Oxaloacetate (ATP used)
Lactate (NADH used)
Acetyl-CoA/CO2 (In presence of O2)
Isoenzymes of Lactate dehydrogenase are used in detecting the following diseases: (4)
Cardiac (myocardial and pulmonary infarctions)
Hepatic (hepatitis and cirrhosis)
Skeletal muscle (hypoxia and muscle trauma)
Neoplastic disorders: leukemias and large tumors
Pyruvate dehydrogenase requires what 5 coenzymes in order to function?
Thiamine pyrophosphate
Lipoic acid
CoA
FAD
NAD+
Enzyme responsible for the isomeration of dihydroxyacetone phosphate (DHAP) and glyceraldehyde 3-phosphate (GAP).
Triose phosphate isomerase
Two possible way to ways to rexoidize NADH -> NAD+
ETC in the presence of oxygen

Pyruvate + NADH <-> Lactate + NAD+
Possible outcomes of pyruvate metabolism
Alanine (carrier of amino groups from muscle to liver)

Oxaloacetate (used to replenish TCA cycle or in gluconeogenesis)

Lactate

Acetyl-CoA (Used in TCA cycle or for fatty acid synthesis)
End products of glycolysis
2 ATP
2 NADH
2 Pyruvate
Low blood pH (high lactate) inhibits which glycolytic enzyme?
Phosphofructokinase
High levels of ATP inhibit which 2 glycolytic enzymes?
Phosphofructokinase
Pyruvate kinase
Sources of Acetyl-CoA
Plamitate (Fatty acid)
Acetoacetate (ketone body)
Pyruvate
Ethanol
The conversion of GTP and ATP is catalyzed by what enzyme?
nucleoside diphosphate
In the brain, this TCA intermediate is converted into the neurotransmitter GABA
a-ketoglutarate
During fasting, this TCA intermediate is transported into cytosol and used in gluconeogenesis.
Malate
This TCA intermediate is used in fatty acid synthesis
Citrate
This TCA intermediate is used to form heme
succinyl CoA
These 2 TCA intermediates can be used for amino acid synthesis
Oxaloacetate and a-ketogluterate
Pyruvate carboxylase is activated by _________.
Acetyl CoA
This enzyme forms oxaloacetate from pyruvate
Pyruvate carboxylase
The oxidation of one NADH yields roughly how many ATP?
~2.5
The oxidation of one FADH2 yields roughly how many ATP?
~1.5
Mitochondrial transport protein that transports ADP from cytosol and ATP to cytosol
Adenine nucleotide translocase (ANT)
Mitochondrial transport protein that transport Pi into mitochondria
Phosphate transporter
This mitochondrial transport shuttle is only active when NADH/NAD ratio is higher in cytosol than in the mitochondria.
malatate-asparate shuttle
In the glycerophosphate shuttle, roughly how many ATP are generated for each NADH oxidized?
~1.5
In the malate-aspartate shuttle, roughly how many ATP are generated for each NADH oxidized?
~2.5
Poision that binds to ATP synthase and blocks H+ channel. Can result in high levels of lactate in blood and urine.
Oligomycin
Two poisons that inhibit complex IV in ETC
Carbon monoxide and cyanide
Poison used as an insect control in gardens and on pets. Inhibits complex I
Rotenone
Poison that inhibits complex I and is used as a sedative and a hypnotic.
Amytal
A fungicide that inhibits complex III
Antimycin A
Tissues/cells that do not use fatty acids during fasting as an energy source
Red blood cells (no mitochondria)
Adipocytes
Brain
The process of adipose tissue being degraded into triglycerides during a fasting state is stimulated by:
elevated glucagon/insulin ration and high epinephrine levels
Fatty acids released from the adipose tissue are bound to _________ and delivered to target tissues
albumin
Degradation of fatty acids mainly occurs where?
mitochondria
In order to be metabolized, fatty acids have to be converted to _________.
Fatty acyl CoA
Fatty acyl CoA can be converted to: (3)
Energy (most tissues, liver)
Storage - Triacylglycerols (adipocytes)
Membrane lipids
Rate limiting enzyme for transporting long chain fatty acids into the mitochondria
Carnitine palmitoyl transferase I (CPTI)
Storage site for most carnitine in the human body
Skeletal muscle
During B oxidation of long chain fatty acids, what is generated during every cycle?
FAD(2H), NADH, Acetyl CoA
Number of ATP generated from one Acetyl CoA in the TCA cycle
10
Where does w oxidation take place?
ER
The main difference between peroxisomal degradiation of fatty acids and beta oxidation is:
No FAD(2H) production in peroxisomal degradition
The 3 ketone bodies
Acetoacetate
B-Hydroxybutyrate
Acetone (not used physiologically)
Liver enzyme responsible for converting glucose 6-phosphate to glucose. This enzyme is not present in muscle cells.
Glucose 6-phosphatase
Chain elongation of glycogen is catalyzed by what enzyme?
glycogen synthase
Glycogen branching enzyme
4:6 transferase
Regulated enzyme in glycogenolysis
glycogen phosphorylase
What part of the cell is glucose 6-p converted to glucose?
ER
In the liver, after glucose 6-p is converted to glucose, the glucose is transported into the cytosol via which transporter?
GLUT-7
Glucose is transported out of liver cells into circulation via which transporter?
GLUT-2
Positive allosteric regulator of glycogen synthesis.
Glucose 6-Phosphate
Allosteric regulators of glycogen degradation
Low glucose (liver only)
High AMP (Muscle only, generated during exercise)
Low glucose 6-phosphate
Low ATP
Glycogen degradation is activated by phosphorylation of ________
Glycogen phosphorylase
Glycogen synthesis is inhibited by phosphorylation of ________
Glycogen synthase
Epinephrine acts through what 2 receptors?
B2-adrenergic
A1-adrenergic
The main activator of glycogenolysis and glycolysis in exercising muscle
AMP
The most sensitive reflection of energy need in skeletal muscle during exercise.
Changes in AMP levels
The major regulated step of beta oxidation in muscle
Carnitine-palmitoyl transferase-1 (CPT-1)
Allosteric inhibitor of Carnitine-palmitoyl transferase-1 (CPT-1)
Malonyl CoA
In fasting conditions, high levels of ______ and _____ in cardiac muscle will inhibit glycolysis.
acetyl CoA
Citrate

(PFK-1 and PDH are inhibited)
2 Enzymes lacking in muscle that prevent gluconeogenesis
glucose 6-phosphatase
pyruvate carboxylase
Symptoms for lactate dehydrogenase deficiency
Muscle cramping and myoglobinuria after intense exercise
Sources of lactate
RBCs
Lens and cornea
Skin
Skeletal muscle
How much ATP is required to form a glucose molecule from lactate?
6
Enzyme that catalyzes Alanine -> Pyruvate
Alanine aminotransferase
Deaminated amino acids used in gluconeogenesis
Alanine -> Pyruvate
Aspartate -> Oxaloacetate
Glutamate -> a-ketogluterate
Pathway of glycerol in gluconeogenesis
Glycerol -> Glycerol 6-phosphate -> Dihydroxyacetone phosphate
Symptoms of biotinidase deficiency
seizures
hypotonia
breathing problems
delayed development

If not treated:
hearing loss
vision loss
alopecia (hair loss)
ataxia
skin rashes
candidiasis
Allosteric inhibitor of fructose-1,6-bisphosphatase
Fructose-2,6-bisphosphate

(Product of PFK-2: F6P -> F2,6BP)
Decrease in fructose-2,6-bisphosphate causes:
Inhibition in glycolysis
Activation of gluconeogenesis
Activation of lipolysis
Increase in fructose-2,6-bisphosphate causes:
Glycogenesis
Glycolysis
Lipogenesis
Increased acetyl-CoA in the liver causes:
Increased gluconeogenesis
Inhibition of pyruvate dehydrogenase
Allosteric activation of pyruvate carboxylase
Vitamin B1 (thiamine) levels are assessed byt the activity of ________________.
Red blood cell transketolase.
Major uses of NADPH
Fatty acid synthesis
Cholesterol synthesis
Steroid synthesis
Nitric oxide synthesis
Deoxyribonucleotide synthesis
Defensive utilization of NADPH
Protection against oxidative damage (erythrocytes)

Detoxification of xenobiotics

Superoxide synthesis
Enzyme that generates superoxide in the phagolysosome to destroy bacteria.
NADPH Oxidase
To synthesize glucose (gluconeogenesis), the liver can use what?
Amino Acids
Glycerol
Lactate