Ch 22 - Oxidation Of Fatty Acids: Ketogenesis Flashcards
Each step in fatty acid oxidation involves _______________, is catalyzed by separate enzymes, utilizes ____ and _____ as coenzymes, and generates ATP.
Acyl-CoA derivatives
NAD+ and FAD
It is an aerobic process, requiring the presence of oxygen.
Fatty acid oxidation
_____________ is a characteristic of starvation and of diabetes mellitus and leads to increased ketone body production by the liver (ketosis).
Increased fatty oxidation
Because gluconeogenesis is dependent upon fatty acid oxidation, any impairment in fatty acid oxidation leads to _________.
Hypoglycemia
This occurs in various states of CARNITINE DEFICIENCY, or deficiency of essential enzymes in fatty acid oxidation, for example _______________ or inhibtion of fatty acid oxidation by poisons, for example ___________.
Carnitine palmitoyltransferase
Hypoglycin
Oxidation of fatty acids occurs in ___________.
Mitochondria
Fatty acids are transported in the blood as ______________.
Free fatty acids
Free fatty acids (FFAs) also called __________ or _________ are fatty acids that are in the UNESTERIFIED STATE.
Unesterified (UFA) or nonesterified (NEFA) fatty acids
In plasma, longer chain FFA are combined with _______ and in the cell they are attached to a FATTY ACID BINDING PROTEIN so that in fact they are never really “free”.
Albumin
____________ are more WATER-SOLUBLE and exist as the UNIONIZED ACID or as a FATTY ACID ANION.
Shorter chain fatty acids
Fatty acids are activated before being _________.
Catabolized
True or False:
Fatty acids must be first converted to an active intermediate before they can be catabolized.
True
This is the only step in the complete degradation of a fatty acid that requires energy from ATP.
Fatty acids are activated before being catabolized
In the presence of ATP and coenzyme A, the enzyme ____________ catalyzes the conversion of a fatty acid to an “active fatty acid” or ____________ using one high-energy phosphate and forming AMP and PPi.
Acyl-coA synthetase (thiokinase)
Acyl-CoA
The PPi is hydrolyzed by ___________ with the loss of a further high-energy phosphate ensuring that the overall reaction goes to completion
Inorganic pyrophosphatase
Found in the endoplasmic reticulum, peroxisomes and inside and on the outer membrane of mitochondria
Acyl-CoA synthetases
Long-chain fatty acids penetrate the inner mitochondrial membrane as _____________.
Carnitine derivatives
Is widely distributed and is particularly abundant in muscle.
Carnitine (B-hydroxy-y-trimethylammonium butyrate)
True or False: Long-chain acyl-CoA cannot penetrate the inner membrane of mitochondria.
True
Located in the outer mitochondrial membrane, transfers long-chain acyl group from CoA to carnitine forming __________ and releasing CoA.
Carnitine palmitoyltransferase-I
Acylcarnitine
Acylcarnitine is able to penetrate the inner membrane and gain access to the B-oxidation system of enzymes via the inner membrane exchange transporter _________________.
Carnitine-acylcarnitine translocase
The acyl group is then transferred to CoA so that acyl-CoA is reformed and carnitine is liberated. This reaction is catalyzed by ____________ which is located on the inside of the inner membrane
Carnitine palmitoyltransferase II
In the ____________, two carbons at a time are cleaved from acyl-CoA molecules starting at the CARBOXYL END.
B-oxidation pathway
The two-carbon units formed are acetyl-CoA thus __________ forms eight acetyl-CoA molecules.
Palmitoyl-CoA
The B-oxidation cycle generates ________ and _______.
FADH2 and NADH
Several enzymes known collectively as __________ are found in the mitochondrial matrix or inner membrane adjacent to the respiratory chain.
Fatty acid oxidase
In the process, large quantities of the reducing equivalents FADH2 and NADH are generated and are used to form ATP by _____________.
Oxidative phosphorylation
The first step is the removal of TWO hydrogen atoms from the 2(a) and 3(b) carbon atoms, catalyzed by __________ and requiring ______. This results in the formation of 🔼2-trans-enoyl-CoA and FAD.
Acyl-CoA dehydrogenase
FAD
The reoxidation of FADH2 by the respiratory chain requires the meditation of another flavoprotein termed _____________.
Electron-transferring flavoprotein
Water is added to saturate the double bond and form 3-hydroxy-acyl-CoA, catalyzed by _______________.
🔼2-enoyl-CoA hydratase
The 3-hydroxy derivative undergoes further dehydrogenation on the 3-carbon cataylzed by _______________ to form the corresponding 3-ketoacyl-CoA compound. In this case, _____ is the coenzyme involved.
L (+) - 3-hydroxyacyl-CoA dehydrogenase
NAD+
Finally, 3-ketoacyl-CoA is split at the 2,3-position by _________ forming acetyl-CoA and a new acyl-CoA two carbons shorter than the original acyl-CoA molecule.
Thiolase (3-ketoacyl-CoA-thiolase)
The shorter acyl-CoA formed in the cleavage reenters the oxidative pathway at __________.
Reaction 2
For example, after SEVEN cycles the C16 fatty acid, _________ would be converted to eight acetyl CoA molecules.
Palmitate
Since acetyl-CoA can be oxidized to CO2 and water via the ______________, the complete oxidation of fatty acids is achieved.
Citric acid cycle
Oxidation of a fatty acid with an ODD NUMBER of carbon atoms yields ACETYL-CoA plus a molecule of ___________.
Propionyl-CoA
Fatty acids with an odd number of carbon atoms are oxidized by the pathway of B-oxidation described above producing acetyl CoA until a three-carbon __________ residue remains.
Propionyl-CoA
This compound is converted to __________, a consistuent of the citric acid cycle.
Succinyl-CoA
Hence, the propionyl residue from an odd-chain fatty acid is the only part of a fatty acid that is _________.
Glucogenic
Oxidation of fatty acids produces a large quantity of ______.
ATP
Transport of electrons from FADH2 and NADH via the respiratory chain leads to the synthesis of ____ high-energy phosphates for each of the seven cycles needed for the breakdown of the C16 fatty acid, palmitate to acetyl-CoA. (7 x 4 = 28)
4
A total of ___ mol of acetyl-CoA is formed and each gives rise to ___ mol of ATP on oxidation in the CTA making ___ mol.
8
10
80
Two must be substracted for the initial activation of the fatty acid, yielding a net gain of ____ mol of ATP per mole of palmitate or 106 x 30.5 = 3233 kJ. This represents ___% of the free energy of combustion of palmitic acid.
106 mol
33%
_______ oxidize very long chain fatty acids.
Peroxisomes