What is the product of glycolysis in red blood cells and exercising muscle?
Lactate
A modified B oxidation leads to the formation of acetyl-CoA and H2O2 and occurs in what organelle?
Peroxisome
Which reducing equivalents are generated and used to form ATP during B oxidation of fatty acids?
FADH2 and NADH
What do adipose TAGs supply during a fasting state?
Fatty Acids
Why can red blood cells use only glucose as a form of energy?
They lack mitochondria
What compounds are used in gluconeogenesis as carbon sources
Lactate, glycerol, amino acids
During starvation, carbohydrates are depleted and free fatty acids are mobilized, which leads to what process?
Ketosis
Urea is formed from amino acids by which organ?
Liver
The last step in the B oxidation of fatty acids involves the conversion of 3-ketoacyl-CoA to acyl-CoA and acetyl-CoA by which enzyme?
Thiolase
In a state of starvation, what organ increases it use of ketone bodies as a major energy source?
Brain
When glucose is produced from non-carb compounds, it is called what?
Gluconeogenesis
What part of TAG is converted to glucose during periods of prolonged fasting?
Glycerol
The catabolism of fatty acids requires what form of energy?
ATP
In the B oxidation of fatty acids, carbons are cleaved from acyl-CoA molecules on which end?
Carboxyl
What are the insulin and glucagon levels of a person in a basal state?
Insulin low, glucagon high
What enzyme is used to form acylcarnitine?
Carnitine palmitoyltransferase-I (CPT-I)
The formation of ketone bodies primarily occurs in what organ?
Liver
After at least one day of fasting, what is the sole source of blood glucose?
gluconeogenesis
After how many days of fasting does the body begin producing ketone bodies for fuel?
3 days
How many mols of ATP are produced in the citric acid cycle for every 8 mol of acetyl-CoA formed?
10
What are the three types of ketone bodies?
B-hydroxybutyrate, acetoacetate, acetone
Which amino acid remains in the muscles in a state of starvation
Leucine
When partially digested food enters the intestine, the hormone ______________ is secreted by the intestine, which signals the gallbladder to contract and release bile acids, and then the pancreas to release digestive enzymes.
Cholecystokinin
Laboratory results for a patient with uncontrolled Type I diabetes mellitus reveal hyperglycemia (634 mg/dL) and hypertriglyceridemia (498 mg/dL). The most likely cause of the hypertriglyceridemia in this patient is which of the following?
Decreased lipoprotein lipase activity
Bile slats must reach a particular concentration within the intestinal lumen before they are effective agents for lipid digestion. This is because of which of the following?
The bile salts solubility in the lumen is a critical factor
What are the components of chylomicrons?
Chylomicrons are formed by triglycerides, phospholipids, cholesterol and apoB48
The apoproteins B-48 and B-100 are similar with respect to which of the following?
They are synthesized from the same gene
LCAT is a plasma enzyme and its substrate is cholesterol contained in _____.
HDL
Elevated serum TG levels can occur due to a number of factors. which one of the following factors reduces TG levels?
Under production of apo B-100
The major enzyme that digests dietary TAGs is ______________ produced in the pancreas. Pancreatic lipase is secreted along with another protein, colipase, in response to the release of the cholecystokinin from the intestine.
Lipase
Which cells produce LPL?
Adipose, Muscle, and Lactating mammary gland cells
Which hormone stimulates the synthesis of lipoprotein lipase?
Insulin
What is the starting point for the LCAT pathway?
Lecithin
In a fasting state, what is released from adipose stores for gluconeogenesis?
Fatty acids and glycerol
What is the optimal pH for digestive enzymes in the intestine?
6
What protein activates lipoprotein lipase?
Apoprotein CII
Chylomicron remnants are taken up by which receptors found in the liver?
Apoprotein E receptors
Which apoprotein is responsible for activating the LCAT pathway?
ApoAI
Which apoprotein inhibits lipoprotein lipase?
ApoCIII
Which of the following is produced in the blood?
LDL
Which is the largest of the lipoproteins?
Chylomicrons
In the LCAT pathway, what is cholesterol converted to?
Cholesterol ester
Fatty acids released from triacylglycerols must form complexes with what molecule to travel in the blood?
Albumin
The synthesis of chylomicrons occurs in which intestinal cellular organelle?
Smooth ER
Where are chylomicrons secreted?
Lymph
What is lipogenesis stimulated by to convert glucose to fatty acids?
Insulin
Which apoprotein is a ligand for the LDL receptor?
ApoB100
What lipoprotein contains triacylglycerol, apolipoprotein B-48, phospholipids, and cholesterol esters?
Chylomicron
Chylomicron remnants are taken up by which receptors found in the liver?
Apoprotein E receptors
What is lipogenesis stimulated by to convert glucose to fatty acids?
Insulin
Lingual and gastric lipases preferentially hydrolyze fatty acid chains of what size?
Short and medium chains
How long after the start of a meal do nascent chylomicrons enter the bloodstream?
1 to 2 hours
The high solubility of cholesterol in blood is due to plasma lipoprotein(s).
VLDL and LDL
What percent of cholesterol in plasma lipoproteins exists in the form of cholesterol esters?
70%
What percent of the cholesterol in plasma exists in the form of free cholesterol?
30%
Bile becomes a very efficient detergent when conjugated to:
Amino acids (glycine and taurine)
Can cholesterol be metabolized to CO2 and water in humans?
No
Which one of the following conditions can induce the formation of gallstones?
Excess HMG-CoA reductase activity, Reduced levels of cholesterol & a-hydroxylase, High levels of deoxycholate that inhibit CYP7A1 enzyme activity (all of the above)
The cholesterol pool of the body is derived from the absorption of dietary cholesterol and biosynthesis primarily in:
Liver and intestine
You decide to treat a patient who has very high levels of serum cholesterol with the statin drug Lipitor (atorvastatin). You know that this drug acts in the metabolic pathway leading to the synthesis of cholesterol. The substrate for the enzyme inhibited by the statin d
HMG-CoA
The initial and rate-limiting step of bile synthesis is achieved by:
7 a-hydroxylase, CYP7A1
Excretion of cholesterol is by way of the liver and gallbladder through the intestine in the form of bile acids
True
Which disease is a result of abnormal, deficient, or inactive LPL?
Familial lipoprotein lipase deficiency
HDL transfers which proteins to chylomicrons and VLDL?
ApoE and apoCII
How does HDL transport cholesterol or cholesterol esters to the liver?
CETP
HDL converts cholesterol to cholesterol esters via which pathway?
LCAT
High levels of which cholesterol are especially atherogenic?
LDL
Which of the following diseases is believed to be both genetic and environmental?
Hypercholesterolemia
How is cholesterol packaged when in the intestines?
Chylomicrons
What is the precursor for cholesterol synthesis?
Acetyl-CoA
Which lipoprotein returns dietary lipids to the liver?
Chylomicron remnants
What adds stability to the phospholipid bilayer of membranes?
Cholesterol
What occurs to the LDL receptors in people with familial hypercholesterolemia?
Lack LDL receptor protein
A majority of synthesized cholesterol is secreted from which type of cell?
Hepatocytes
Which disease leads to premature coronary heart disease due to atherosclerosis and thrombosis due to the inhibition of fibrinolysis?
Familial lipoprotein A excess
Which proteins are responsible for moving unwanted or excess cholesterol from enterocytes to the gut lumen?
ABCG5 and ABCG8
What enzyme is responsible for releasing free fatty acids for milk production?
LPL
What member of the ABC family is required for reverse cholesterol transport and HDL biogenesis?
A1
The presence of a fatty streak is early gross evidence of what type of formation?
Atherosclerotic plaque
What do nascent VLDL particles accept from HDL particles to form mature VLDL particles?
Apolipoproteins
What does VLDL convert to initially?
IDL
After digestion by lipase, LDL returns to the liver and enters cells via what process?
Endocytosis
Cholestyramine leads to excretion of what product?
Bile acids
The second pathway of biosynthesis and degradation of bile acids may occur in what organelle?
Mitochondria
What structural part of bile salts makes them well suited for micelle formation?
Hydrophobic, hydroxyl group
Which of the following is a primary bile acid?
Taurocholic acid
What forms micelles with fatty acids released from dietary triglycerides?
Bile salts
Which of the following is a secondary bile acid?
Lithocholic acid
What effect does rising levels of cholesterol and bile acids have on HMG-CoA reductase?
Proteolysis susceptibility
What does the lipase/colipase complex promote the degradation of?
Triacylglycerol
Bile acids improve the absorption of which vitamins?
A,D,E,K
Where does fat emulsification and micelle formation occur?
Small intestine
How does cholestyramine lower cholesterol levels in the blood?
Binds bile acids to limit reabsorption
Once micelles transport degradation products to the mucosa, what occurs to the products?
Reassembly into triacylglycerols
The production of secondary bile acids occurs in which organ?
Intestines
In what organ is bile synthesized?
Liver
Primary and secondary bile acids are absorbed in the intestines and returned to which organ?
Liver
The presence of bile and fatty acids in the duodenum increases the level of which hormone?
Secretin
What is the rate-limiting step in the synthesis of bile acids?
CYP7A1 reaction
What property do bile acids have that promotes the encircling and degradation of triglycerides and phospholipid fat particles in food?
Detergent
Bile salts generated with α-hydroxyl groups at the 3 and 7 positions form which set of bile salts?
Chenocholate
The conjugation of glycine and taurine occurs in what part of the cell?
Peroxisome
What are the four classes of plasma lipoproteins in normal fasting humans?
HDL, LDL, IDL, VLDL
The protein component of a lipoprotein particle is called
apolipoproteins
True or False: The HDL particle has higher percentage of protein than VLDL particle
True
True or False: VLDL is synthesized in the intestine and liver
False
Lipoprotein lipase is located on the surface of-
endothelial cells of capillaries
Which apo protein is required for activation of lipoprotein lipase?
ApoC-II
The pKa of the bile acids is approximately 6. The intestinal lumen has a pH of 6. That means-
Half of the bile molecules are present in the protonated (acid) and the other half are ionized (salt).
When bile salts form conjugates with taurine or glycine their pKa values decrease to as low as 2. What would you expect to see in the intestinal lumen that has a pH of 6?
Majority of bile molecules are in ionized form (salt)
What role does intestinal bacteria play regarding bile salt molecules?
Deconjugate and Dehydroxylate bile salts
Which type of secondary bile salt/acid has the least solubility in the intestinal lumen and its major fate is excretion?
Lithocholic acid
What is the rate of HMG-CoA reductase mRNA synthesis controlled by?
SREBP
The synthesis of all steroid hormones begins with the conversion of cholesterol to what compound?
Progesterone
All the carbons of cholesterol are derived from which precursor?
Acetyl-CoA
SREBPs are integral proteins of what cellular organelle?
Endoplasmic reticulum
How many condensed molecules of acetyl-CoA are required to form HMG-CoA?
3
How do statins lower cholesterol?
Inhibit HMG-CoA reductase
In what part of the cell does cholesterol synthesis occur?
Cytosol
At what point is the SREBP:SCAP complex transported to the Golgi apparatus?
decrease in cholesterol
What are the three main organs, aside from the liver, in which significant quantities of cholesterol are biosynthesized?
Gut, adrenal cortex, gonads (ballz)
How does hyperinsulinemia change the molecule of HMG-CoA reductase?
Leads to dephosphorylation
What effect does increased glucagon levels have on HMG-CoA reductase?
Inactivation
How many fused cyclic rings does the cholesterol molecule contain?
4
What is the precursor for glucocorticoids, androgens, and estrogens?
Cholesterol
The conversion of HMG-CoA to mevalonic acid is catalyzed by which enzyme?
HMG-CoA reductase
What class of steroid hormones contains 21 carbons?
Glucocorticoids
Two molecules of farnesyl pyrophosphate undergo a fusion reaction to yield what compound?
Squalene
Cholesterol is stored as cholesterol esters in the cells of what organ?
Adrenal cortex
What is the energy source for cholesterol synthesis?
ATP
In order for mevalonate to form, what compound must be reduced via catalyzation?
HMG-CoA
The synthesis of what compound is considered the rate-limiting step in cholesterol formation?
Mevalonate
What type of bond helps nucleotide molecules form RNA and DNA strands?
Phosphodiester
Gout is a metabolic disorder of which of the following?
Purine catabolism
The uric acid pathway occurs in what organ?
GI tract
What is the end product of purine catabolism in humans?
Uric acid
Why does abnormal catabolism of pyrimidines not form as many serious diseases as catabolism of purines?
Pyrimidines form highly water soluble products
Which of the following is produced by the catabolism of pyrimidines?
CO2
How many ring structures does adenine have?
2
What is formed from ribose, glycine, aspartate, glutamine, tetrahydrofolate, and CO2?
IMP
Disorders of β-alanine and β-aminoisobutyrate metabolism arise from defects in which enzymes?
Pyrimidine catabolism
Which component of nucleosides and nucleotides is important in energy storage and signaling?
Phosphate group
Which purine molecule is converted into uric acid and excreted in the urine?
Xanthine
Which molecules have a pyrimidine ring in their structures?
Cytosine, thymine and uracil
Adenosine is converted to what compound via adenosine deaminase in the uric acid pathway?
Inosine
Which molecules have a purine ring in their structures?
Adenine and guanine
Nucleotides are similar in structure to nucleosides except for the addition of what molecule?
Phosphate
What medical conditions could result from mistakes or deficiencies in nucleosides or nucleotides?
Death
How many ring structures does thymine have?
1
One of the many functions of nucleosides and nucleotides includes which of the following?
Maintaining/transferring genetic information
Which molecule has a nitrogenous base and a five-carbon carbohydrate?
Nuceloside
How does allopurinol work as a treatment for gout?
Reduces production of uric acid
What enzyme is activated by increased glutamate, N-acetylglutamic acid, and/or arginine concentrations?
Carbamoyl phosphate synthetase
Conversion from citrulline to argininosuccinate requires?
a single ATP molecule is required for the reaction of citrulline to argininosuccinate
Glutamate donates the first amino group for urea; which molecule donates the second?
Aspartate
Nitrogen-based waste products are excreted as what compound?
Urea
Which of the following is an essential amino acid?
Lysine
Amino acids are metabolized via which pathway?
Citric acid cycle, glycolysis
The enzyme argininosuccinate lyase is used during which reaction?
Argininosuccinate to arginine
The reaction of CO2 and NH4 is catalyzed by CPS-I and forms carbamoyl phosphate in the urea cycle. Where does this reaction occur?
Mitochondria
The urea cycle occurs mostly in what bodily organ?
Liver
What characteristic of amino acids makes them useful for making a variety of fuels for the body?
Carbon skeleton
How many ATP molecules are required in the urea cycle?
3
What does glutamate donate for the conversion to urea?
NH3
The production of urea occurs in what part of the cell?
Cytosol
The conversion of what molecule allows for the utilization, generation, and/or storage of ammonia?
Α-ketoglutarate to glutamate
What does carbamoyl phosphate combine with to form citrulline?
Ornithine
What is used as building blocks for proteins, nucleic acids, hormones, neurotransmitters, antioxidants, and signaling molecules
Amino Acids
Which of the following is in the correct order for the urea cycle?
Carbamoyl phosphate, citrulline, argininosuccinate
What does α-ketoglutarate convert to?
Glutamate
How many amino groups are used for the conversion of products to urea?
2
What is the function of Argininosuccinate synthetase?
Conversion from citrulline to arginiosuccinate
Urea is produced in the
Liver
Which amino acid donates the second nitrogen atom during the Urea Cycle?
Aspartate
Ammonia formed in muscle and other organs is transported through the blood circulation in nontoxic form as-
Glutamine and Alanine
Fumarate from urea cycle is a source for-
Glucose, Aspartate, ATP energy
How is the urea cycle regulated?
by CPS-I
A 9-month-old infant was admitted to the hospital in a coma and a temperature of 103°F. His pulse was elevated, his liver was enlarged, and an electroencephalogram was grossly abnormal. Intravenous glucose was administered. He improved rapidly and came out of the coma in 24hrs. Analysis of his urine showed abnormally high amounts of glutamine, which suggested a high blood ammonium ion concentration.
Which enzyme may be defective in this patient?
Glutaminase
The most important amino acids as a source of fuel that generate a large amount of NADH and FADH2 are:
Valine, Leucine, and Isoleucine
The amount of fumarate used to form ATP is approximately equal to that required for the urea cycle and gluconeogenesis, meaning that-
The liver gains no net energy
Why do caloric restriction and low blood glucose increase the activities of glutamate dehydrogenase?
The activity is raised in order to increase the amount of a-ketoglutarate produced, which can be used to provide energy.
(True or False) Any urea that enters the intestinal tract is cleaved by urease-containing bacteria in the intestinal lumen and the ammonia is absorbed back into the body
True