Beta Oxidation

Question 1

Site of beta oxidation of FA

Answer 1

Mitochondria; Aerobic

Question 2

Beta oxidation generates large quantities of ATP

Answer 2

106

Question 3

At High rate of Fatty Acid oxidation

These are produced

Answer 3

Acetoacetate
3-Hydroxybutarate
Acetone

Question 4

Most fats in the body are stored as

Answer 4

TAG

Question 5

Glycerol + 3 FA

Answer 5

TAGS

Question 6

The only step that requires ATP

Through the enzyme acyl coA synthetase (thiokinase)

Answer 6

Fatty acids are activated first before they can be metabolized

Question 7

Acyl CoA undegoes beta oxidation to become

Answer 7

Acetyl CoA

Question 8

Fatty acid is converted to Acyl CoA

Acyl CoA is converted into

by the enzyme

Answer 8

Acyl Carnitine

Carnitine Palmitoyl Transferase I

Question 9

Acyl carnitine is shuttled from cytosol to

Answer 9

mitochondria

Question 10

AcylCarnitine is converted to

by the enzyme

Answer 10

Acyl CoA

Carnitine Palmitoyl Transferase enzyme II

Question 11

Acetyl CoA can be

Answer 11

Recycled to become
Acyl CoA
Proceed to Krebs

Question 12

Carnitine transports

Answer 12

Long chain fatty acids through the inner mitochondrial membrane

Question 13

Transports long chain fatty acid through inner mitochondrial membrane

Only the metabolic product is converted can be transferred into mitochondrial membrane

Answer 13

Carnitine

Acylcarnitine

Question 14

Inside the inner mitochondrial membrane acylcarnitine reacts with CoA catalyzed by

Answer 14

CPTII

Question 15

Step 1 of beta oxidation: long chain fatty acid is

via enzyme

Answer 15

Dehydrogenated

fatty acyl CoA dehydrogenase

Question 16

Step 2 of Beta oxidation

Trans-delta2-enoyl CoA is

Answer 16

hydrated

Question 17

Step 3 Beta Oxidation

L-B hydroxylacyl CoA is

by enzyme

Answer 17

dehydrogenated again

B hydroxyl CoA dehydrogenase

Question 18

Step 4 of beta oxidation:

by the enzyme

Answer 18

Thiolysis

Thiolase enzyme

Question 19

Steps of Beta Oxidation

Answer 19

Dehydrogenation
Hydration
Oxidation
Thiolysis

Question 20

Beta oxidation invovles

Answer 20

FA oxidation

Shortening of B Acyl CoA Chain

Question 21

Disorder of fatty acid synthesis involves this organelle

Answer 21

Peroxisome

Question 22

Very long chain FA are associated with this organelle

Answer 22

peroxisomes

Question 23

Catalyzes first oxidative step of pathway

Answer 23

Acyl coA catalase ??

Question 24

Beta oxidation is induced by

Answer 24

High fat diets
Hypolipidemic drugs (Clofibrate)

Question 25

Newborn, preterm

Answer 25

Carnitine deficiency Tx: oral supplementation with Carnitine

Question 26

Liver Reduced FA oxidation Ketogenesis Hypoglycemia

Answer 26

CPT I deficiency

Question 27

Skeletal muscle | Liver

Answer 27

CPT II deficiency

Question 28

Inherited absence of peroxisomes Loss of peroxisimal functions Severe neurological disease Die in first year of life

Answer 28

Zellweger’s cerebrohepatorenal syndrome

Question 29

Enzyme deficient in Zellweger’s

Answer 29

Pipicolate oxidase

Question 30

Rare neurologic disorder Accumulation of phytanic acid Pathologic effect on membrane function, protein prenylation and gene expression

Answer 30

Refsum’s disease | Phytanic acid storage disease

Question 31

Enzyme deficiency in Refsum’s disease

Answer 31

Phytanoyl CoA hydroxylase | Peroxin

Question 32

Gene mutation in Refsum disease

Answer 32

PEX7 Gene

Question 33

Gene mutation in Zellweger Syndrome

Answer 33

PEX1 gene

Question 34

Refsum presents with

Answer 34

Ataxia | Icthyosis

Question 35

Associated with ingestion of the fruit of akee tree Toxin inactivates medium and short chain acting Acyl-CoA dehydrogenase inhibiting beta oxidation and causing hypoglycemia

Answer 35

Jamaican vomiting sickness Hypoglycin toxin

Question 36

Undergoing hemodialysis uses up the body’s L carnitine It is also used as replacement supplement in strict vegetarians, dieters, and low weight or premature infants

Answer 36

L-Carnitine

Question 37

Process by which Fatty acids are broken down into Acetyl Coa to form ketones and metabolic fuel Forms majority of energy available to liver Forms ketone bodies available to brain and muscle Size of fatty acid determines the location of beta oxidation

Answer 37

Beta oxidation of fatty acid

Question 38

Short/Medium Chain | (2-12 carbons) are broken down in

Answer 38

Diffuse freely in mitochondria

Question 39

Long Chain (14-20) Beta oxidation

Answer 39

Must use carnitine transport into mitochondria

Question 40

Very long chain >20 carbons Beta oxidation

Answer 40

Must be oxidized in a peroxisome

Question 41

Step 1: Fatty acids -> Fatty acyl coa by the enzyme

Answer 41

Fatty Acyl Coa Synthetase

Question 42

Step 2 Fatty acyl coa diffuses into mitochondrial membrane and is converted to Fatty acyl carnitine by the enzyme

Answer 42

Carnitine Acyltransferase I

Question 43

Fatty acyl coa -> Fatty acyl-carnitine by Carnitine Acyltransferase I is inhibited by

Answer 43

Malonyl CoA (Fatty acid synthesis)

Question 44

Step 3 Fatty acyl-carnitine is shuttled to the mitochondria and Fatty acyl carnitine -> Fatty acyl coa + Carnitine by the enzyme

Answer 44

Carinitine Acyltransferase 2 | in mitochondria

Question 45

Step 1 FATTY ACID OXIDATION Fattyl Acyl Coa -> Acetyl coa by the enzyme

Answer 45

Fatty acyl coa dehydrogenase or Medium Chain Acyl Coa Dehydrogenase Long Chain Acyl Coa Dehydrogenase

Question 46

Step 2 FA BETA OXIDATION Acetyl coa ->

Answer 46

``` Ketone bodies (liver) Acetyl Coa to TCA ```

Question 47

Rate limiting enzyme of Beta Oxidation of Fatty acid

Answer 47

Carnitine Palmitoyl Transferase-1 | CAT-1

Question 48

No transport of Fatty acyl coa to mitochondrial membrane Hypoketotic Hypoglycemia

Answer 48

Systemic Primary Carnitine Deficiency

Question 49

Myoglobinuria Hypotonia, weak Inc TAG in muscle (from build up of fatty acyl coa) Fatty acyl-carnitine -> Fatty acyl coa + carnitine Carnitine Palmitoyl Transferase 2

Answer 49

Myopathic CAT-2 Deficiency

Question 50

Deficiency of fatty acyl coa dehydrogenase (Non conversion of fatty acyl coa -> acetyl coa) ``` Non ketotic (unable to produce glucose) hypoglycemia Hepatic dysfunction (inc accumulation of fatty acyl coa in liver) Hyperammonemia ``` Avoid fasting

Answer 50

Medium chain Fatty Acyl-CoA Dehydrogenase Deficiency