Questions for TG breakdown, FA oxidation, Ketone metabolism:

1. Anhydrous fat store more than how many times of energy than glycogen?

2. Glycogen and glucose stores provide enough energy to sustain biological function for

how many hours?

3. How long do TGs provide stores for?

4. TG are degraded to fatty acids and glycerol by what enzyme?

5. The free fatty acids are released into what type of tissues?

6.

7. A factor affecting lipolysis is glucose, under adequate glucose intake, what is more

available therefore re-esterfication is more than lipolysis?

8. Reduced bioavailability of glucose increases what type of glycerol?

9. Adipolysis in diabetes mellitus and starvation are better that what type of esterification?

10. Which hormone inhibits lipolysis by decreasing the concentration of cAMP?

11. Increased or decreased phosphorylation inactivates hormone sensitive lipase?

12. Is increased phosphorylation the active or inactive form of the enzyme?

13. What is lipolysis?

14. What is the role of insulin in adipolysis?

15. What is the difference between fatty acids and TG?

16. What is the role of prolactin in adipolysis?

17. What other hormones other than insulin and prolactin stimulate adipolysis?

18. Drugs like caffein and theophylline increase lipolysis by inhibiting what enzyme that

degrades cAMP?

19. What are free fatty acids also called?

20. In plasma, longer chain FFA are combined with what?

21. In the cell, longer chain FFA are combined with what?

22. Shorter chain fatty acids are more water soluble and exist as what?

23. Glycerol formed during TAG degradation turns into what type of product?

24. Glycerol that is formed during TAG degradation cannot be used due to a deficiency of

what?

25. The glycerol formed during TAG is released into the plasma and transported to the liver

for what pathways?

26. Fatty acids can be oxidized by what 4 oxidations?

27. What is beta oxidation?

28. What is alpha oxidation?

29. What is omega oxidation?

30. What is peroxisomal oxidation?

31. In beta oxidation of fatty acids, a saturated acetyl CoA is degraded by a recurring

sequence of what four reactions?

32. To begin beta oxidation, what fatty acids must first be converted to an active intermediate

before they can be catabolized, which enzyme activates it?

33. Activation of a fatty acid for beta oxidation to begins is the only step that requires what

type of energy?

34. What happens during beta oxidation?

35. What is generated by beta oxidation?

36. Fatty acids are activated on the outer mitochondrial membrane, whereas they are oxidized

where?

37. Activated long chain fatty acids are transported across the membrane conjugating them to

what ,a zwitterionic alcohol?

38. Carnitine is widely distributed and is particularly abundant in what?

39. Where is carnitine obtained from?

40. What is the first role of carnitine?

41. What is the second role of carntitine?

42. What are the steps of B-Oxidation?

43. How does palmitoyl CoA get to the ETC?

44. How many ATPs are produced by the complete oxidation of one mol of palmitic acid?

45. 2.5 ATP per NADH= 17.5 (7 cycles of b oxidation)

1.5 ATP per FADH2= 10.5

Total 28 ATP

10 ATP per acetyl CoA=80 acetyl CoA

Total 108 ATP

2 ATP equivalents consumed during activation of palmitate to palmitoyl CoA

Total 108-2 ATP=106 ATP

46. What is carnitine deficiency?

47. Carnitine deficiency or carnitine transferase/translocase produces what type of

symptoms?

48. What is the treatment for carnitine deficiency?

49. Beta oxidation disorder- Nonketotic hypoglycemia is caused by lack of what?

50. Beta oxidation disorder- Jamaican sickness is caused by inactivation of what?

51.

52. B-oxidation from FFA is controlled by the what?

53. Insulin inhibits which oxidation?

54. What stimulates B-oxidation?

55. What is the fate of Acetyl CoA produced from TG breakdown?

56. Odd chain fatty acids are oxidized by the pathway of B-oxidation, producing Acetyl CoA,

until how many propionyl CoA residues remain?

57. The 3 carbon propionyl CoA residues are converted in to what product that is a

constituent of the TCA cycle?

58. Which part of a odd chain fatty acid is only glucogenic?

59. A-oxidation of fatty acids takes place where?

60. What is A-oxidation?

61. What is the biological significance of A-oxidation?

62. What is refsum’s disease (RD)?

63. When does classic refsum disease manifest in children?

64. When does infantile refsum’s disease manifest?

65. What are clinical manifestation of refsum’s disease?

66. What are the laboratory diagnosis of refsum’s disease?

67. What is the treatment for refsum’s disease?

68. What is omega oxidation of fatty acids?

69. The omega oxidative pathway helps which types of states that provide an effective means

for the elimination of toxic levels of free fatty acid?

70. What is peroxisomal oxidation?

71. What is chain shortening in peroxisomes?

72. What is the significance of peroxisomal oxidation?

73. Are ketone bodies water soluble or water insoluble?

74. Ketone bodies can be transported in what form?

75. How are fatty acids released into adipose tissue and converted by the liver?

76. Which substrates are also referred to as ketone bodies?

77. What is the significance of ketone bodies?

78. What is ketogenesis?

79. Where does ketogenesis take place?

80. What enzymes are responsible for ketogenesis to take place?

81. What is the significance of Acetone and B hydroxybutyrate?

82. What is ketolysis?

83. Why are ketone bodies synthesized in the liver but not utilized in the peripheral tissues?

84. What are the steps of ketosis regulation?

85. What happens in the first step of ketosis regulation?

86. What happens in the second step of ketosis regulation?

87. What happens in the third step of ketosis regulation?

88. What is the significance of ketosis?

89. What causes ketosis?

90. What are the key concepts?

1. 6 times
2. 24 hours

3. Several weeks

4. Hormone sensitive lipase

5. Energy requiring

6. -

7. Glycerol P

8. Glycerol P

9. Re-esterfication

10. Insulin

11. Decreased

12. Active

13. Lipolysis is the first step in the fat loss process. Without lipolysis there will be no

released fat that will be oxidized and turned into ATPs. Lipolysis is important because it

is the process which releases the free fatty acids from stored fats in adipocytes to the

blood stream. Is the metabolic pathway through which lipid triglycerides are hydrolyzed

into a glycerol and free fatty acids.

14. Promotes glucose utilization, which increases availability of glycerol-P, NADH, acetyl

CoA. It helps create increased fatty acid and TG synthesis

15. TG are 3 fatty acids that went through esterification. Fatty acids make up the TG
16. Large concentration of Prolactin shows the similar effects of insulin

17. Catecholamines, growth hormone, corticosteroids, ACTH, alpha/beta MSH, TSH, and

vasopressin

18. Phosphodiesterase enzyme

19. Unesterfied UFA or nonesterfied NEFA

20. Albumin

21. Fatty acid binding protein

22. Unionized or as fatty acid ion

23. Waste

24. Glycerol kinase enzyme

25. Gluconeogenesis or oxidized through the pathway of glycolysis

26. Beta/Alpha oxidation, omega oxidation, peroxisomal oxidation

27. Major mechanism that occurs in the mitochondria matrix, 2 C units are released as acetyl

CoA per cycle.

28. Predominantly takes place in the brain and liver, one carbon is lost in the form of CO2

per cycle.

29. Minor mechanism but becomes important in conditions of impaired beta oxidation.

30. Mainly for the trimming of very long chain fatty acids.

31. Oxidation by FAD/dehydration, hydration, oxidation/dehydrogenation by NAD+, and

thiolysis by CoASH

32. Acyl Co synthetase

33. 2 ATP

34. The fatty acyl chain is shortened by two carbon atoms as a result of the chain broken

between a(2) -and b(3) carbon atoms

35. FADH2,NADH, Acetyl CoA

36. Mitochondrial matrix

37. Carnitine

38. Muscle

39. Foods, particularly animal based foods, and via endogenous synthesis

40. An acyl group is transferred to the hydroxyl group of carnitine to form acyl carnitine, this

reaction is catalyzed by carnitine acyl transferase 1 to be shuttles across the inner

mitochondrial membrane by translocase.

41. The acyl group is transferred back to CoA on the matrix side of the membrane, This

reaction is catalyzed by carnitine acyl transferase 2. And then translocase returns

carnitine to the cytosolic side in exchange for an incoming acyl carnitine.

42. Fatty acyl CoAtrans fatty enoyl CoA hydroxy acyl CoA b-ketoacyl CoA fatty

acyl CoAacetyl CoA

43. Plamitoyl CoA palmitoloyl CoA (with FAD acyl CoA DH) FAD2 +ETF FAD2

ETF QO CoQ  COQH2

44. 106 ATP

45. –
46. Carnitine deficiency results from inadequate intake of or inability to metabolize carnitine.

Decreased endogenous synthesis of carnitine due to a severe liver disorder, excess loss of

carnitine due to diarrhea, diuresis, hemodialysis, carnitine leak from renal tubules

(primary carnitine deficiency)

47. Muscle cramps during exercise, severe weakness and death. Muscle weakness related to

importance of fatty acid as long-term energy source. Hypoglycemia and hypo ketosis.

48. Oral carnitine, avoid fasting and strenuous exercise, linoleic acid, linolenic acid, and

patients with fatty acid oxidation disorder require a high carbohydrate, low fat diet.

49. Mitochondrial medium chain acyl CoA dehydrogenases

50. Inactivation of medium and short chain acyl CoA dehydrogenases inhibiting b oxidation

causing hypoglycemia. (unripe fruit of akee tree contains toxin hypoglycin)

51. –

52. Carnitine palmitoyl transferase 1 gateway into the mitochondria

53. B-oxidation

54. Glucagon
55. TCA, ketogenesis, synthesis of cholesterol, synthesis of steroids, detox of xenobiotics,

synthesis of acetyl choline

56. 3 carbon residues

57. Succinyl-CoA

58. Propionyl residue

59. Microsomes of brain and liver

60. Involves decarboxylation process for the removal of single carbon atom at one time with

the resultant production of an odd chain fatty acid that can be subsequently oxidized by

B-oxidation for energy production. Only aerobic and no prior activation is required.

61. It can oxidate phytanic acid produced from dietary phytol, this acid is significant

constituent of milk lipids and animal fats. The hydroxy fatty acids produced as

intermediates of this pathway like cerebronic acid can be used for synthesis of

cerebrosides and sulfatides, odd chain fatty acids produced upon decarboxylation in this
 pathway can be used for the synthesis of sphingolipids.

62. Neurocutaneous syndrome that is characterized biochemically by the accumulation of

phytanic acid in plasma and tissues. There is deficient activity of phytanic acid oxidase

enzyme catalyzing the first step of phytanic acid alpha oxidation.

63. 2-7 years old but dx is delayed until early adulthood.

64. Early infancy

65. Progressive weakness, foot drop, loss of balance, shortened bones in fingers or toes,

epicanthal folds, flat bridge of nose and low set ears and ichthyosis (dry scaly skin)

66. Blood levels of phytanic acid are increased greater than normal of < 0.2mg/dL to 10mg to

50 mg/dL and accounts for 5 to 30 % of serum lipids. Phytanic oxidase activity

estimation in skin fibroblast cultures, imaging, skeletal radiography to show bone

changes.
67. Eliminate all sources of chlorophyll from the diet, greens, reduce phytanic intake of

50mg/dL to 5mg/dL and plasmapheresis (plasma exchange).

68. Hydroxylation and occurs in the ER of many tissues, hydroxylation takes place on the

methyl carbon at the other end of the molecule from the carboxyl group or on the carbon

next to the methyl end. Hydroxy fatty acids can be further oxidized to a dicarboxylic acid

via sequential reactions of alcohol dehydrogenase and aldehyde dehydrogenases. This

process occurs primarily with medium chain fatty acids.

69. Diabetes, chronic alcohol consumption and starvation

70. A flavoprotein dehydrogenase transfers electrons to O2 to yield H2O2 instead of

capturing the high energy electrons as FADH2, as occurs in mitochondrial beta oxidation.

All other steps are the same as the other oxidations that occur in the mitochondria.
71. Peroxisomal reactions involve shortening of very long chain fatty acids, and conversion

of cholesterol to bile acids and formation of either lipid.

72. The congenital absence of functional peroxisomes, an inherited defect that causes

Zellweger syndrome. The most common features include vison, disturbances, prenatal

growth failure, lack of muscle tone, unusual facial characteristics, mental retardation,

seizures, and inability to suck or swallow.

73. Ketone bodies can be regarded as water soluble.

74. They are transported in the form of acetyl units.

75. Fatty acids are released by adipose tissue and converted into acetyl units by the liver,

which then exports them as ketone bodies.

76. Acetoacetate, D-B hydroxybutyrate, acetone

77. Ketone bodies serve as a fuel for extrahepatic tissues. Liver can synthesize but cannot

utilize the ketone bodies. Brain can utilize but cannot synthesize the ketone bodies, and

only utilizes the ketone bodies only under condition of glucose deprivation.

78. It is the ability to create acetyl CoA as a substrate for precursor for ketone bodies.

79. Ketogenesis takes place solely in the liver.

80. The enzymes responsible for ketone body formation are associated mainly with the

mitochondria Both enzymes HMG CoA synthase and HMG CoA lyase must be present in

mitochondria for ketogenesis to take place.

81. Type 1 diabetes mellitus can be diagnosed in a person with high level of acetoacetate in
the blood. Acetone breath has been used as a crude method of diagnosing this disease.
The ratio of B hydroxybutyrate to acetoacetate depends on the NADH/NAD ratio inside
mitochondria, if NADH ratio is higher, the liver releases a higher proportion of B
hydroxybutyrate.
82. Ketolysis is the reversal of ketone body synthesis. Ketone bodies are utilized by
extrahepatic tissues via a series of cytosolic reactions. Ketones must be reconverted to
acetyl CoA in the mitochondria.

83. The enzyme succinyl CoA, Acetoacetate CoA transferase is also known as thiophorase,
which is present at high levels in most tissues except in the liver. Very low level of
enzyme expression in the liver allows the liver to produce ketone bodies but not utilize
them, this ensures that extrahepatic tissues have access to ketone bodies as a fuel source
during prolonged fasting and starvation. Lack of this enzyme in the liver prevents the
futile cycle of synthesis and break down of acetoacetate.
84. Lypolysis in adipose tissue, fate of fatty acid, and fate of acetyl CoA

85. Ketosis does not occur unless there is an increase in the level of circulating free fatty
acids that arise from lipolysis of triacylglycerol in adipose tissue. When glucose levels
fall, lipolysis induced by glucagon secretion causes increased hepatic ketogenesis due to
increased substrate (free fatty acids) delivery from adipose tissue. Conversely insulin
released in the well fed state inhibits ketogenesis via the triggering of dephosphorylation
and inactivation of adipose tissue hormone sensitive lipase (HSL).
86. Free fatty acids are either oxidized to CO2 or ketone bodies or esterfied to triacylglycerol
and phospholipids. Malonyl CoA, the initial intermediate in fatty acid biosynthesis
formed by acetyl CoA carboxylase in the fed state, is a potent inhibitor of CAT-1. Under
these conditions, free fatty acids enter the liver cell in low concentrations and are nearly
all esterfied to acylglycerols and transported out of the liver in very low density
lipoproteins (VLDL).
87. The acetyl CoA formed in B-oxidation is oxidized in the citric acid cycle, or it enters the
pathway of ketogenesis to form ketone bodies. As the level of serum free fatty acids is
raised, proportionately more free fatty acids are converted to ketone bodies and less are
oxidized via the citric acid cycle to CO2. Entry of acetyl CoA into the citric acid cycle
depends on the availability of oxaloacetate for the formation of citrate, but the
concentration of oxaloacetate is lowered if carbohydrate is unavailable or improperly
utilized.
88. Heart muscle and the renal cortex use acetoacetate in preference to glucose. In contrast,
the brain adapts to the utilization of acetoacetate during starvation and diabetes. In
prolonged starvation, 75% of the fuel needs of the brain are met by ketone bodies. The
synthesis and utilization of ketone bodies is increased in persons eating diets extremely
high in fat and low in carbohydrate, or starving, or suffering from severe lack of insulin
(type 1).
89. Uncontrolled diabetes mellitus, starvation and chronic alcoholism. Von Gierke’s disease,
glycogen storage disease type 6 (due to phosphorylase kinase deficiency), heavy
exercise, low carbohydrate or high fat diet, pyruvate carboxylase deficiency, prolonged
ether anesthesia, toxemia of pregnancy
90. The triacylglycerols are degraded to fatty acids and glycerol by hormone sensitive lipase
(HSL). Except insulin, all other hormones such as catecholamines, growth hormone,
corticosteroids, ATCH, alpha and Beta MSH, TSH, and vasopressin stimulate adipolysis.
Free fatty acids released by adipolysis are made accessible as a fuel in other tissues. Fatty
acids can be oxidized in diverse ways; however mitochondrial B-oxidation is the major
mechanism of oxidation.
Impaired B-oxidation causes non ketotic hypoglycemia. Refsum’s disease occurs due to
impaired Alpha oxidation. Zellweger syndrome results from impaired peroxisomal
oxidation. Ketosis occurs in conditions of glucose deprivation. Ketone bodies are
synthesized in the liver but not utilized in the extra hepatic tissues. Prolonged starvation,
low carbohydrate or high fat diet, heavy exercise, uncontrolled DM, prolonged ether
anesthesia and toxemia of pregnancy are the common causes of ketosis.