2.4.2 Metabolism III

Question 1

What are three of the essential fatty acids?

Answer 1

linoleic, linolenic, arachidonic acids

Question 2

In what two ways does glucose contribute to fatty acid synthesis?

Answer 2

Providing carbons

Feeding the pentose phosphate pathway, which generates NADPH

Question 3

What is the significance of ketogenesis?

Answer 3

Question 4

What does reaction does insulin activate?

Answer 4

Malonyl-CoA -> Palmitate

Activation of Acetyl CoA carboxylase - leading to the formation of malonyl CoA which will inhibit FA breakdown

Ultimately, reducing the formation of ketone bodies

Question 5

What is the enzyme deficiency of farber’s dz? accumulating lipid?

Answer 5

Ceramidase; Acyl-Sphingosine Ceramide

Question 6

What is the enzyme deficiency in Gaucher’s dz? Accumulating lipid?

Answer 6

Beta-glucocerebrocidase; Glucosyl ceramide

Question 7

What is multiple sclerosis? What is lost?

Answer 7

A demyelinating dz; phospholipids (particularly ethanolamine plasmalogen) and sphingolipids of white matter

Question 8

What are the clinical symptoms of niemann-pick dz?

Answer 8

Question 9

What are the clinical symptoms of the three types of gaucher’s dz?

Answer 9

Question 10

What is the enzyme dificiency in Fabry’s Dz? Accumulating Lipid?

Answer 10

alpha-galactosidase; globotricylceramide

Question 11

Under circumstances where significant fatty acid breakdown occurs, what organ will release ketones which can be used by other organs?

Answer 11

Liver

Question 12

Describe the break down of fatty acids into a substrate that will feed into the TCA cycle

Answer 12

Question 13

What is often bound to the individual enzymes of the fatty acid synthase system?

Answer 13

Acyl carrier protein (ACP), which contains pantothenic acid

Question 14

How is ceramide synthesized?

Answer 14

Question 15

Other than citrate, what two substances can regulate Acetyl-CoA Carboxylase?

Answer 15

Glucagon and insulin

Question 16

Where must acetyl CoA be sent in order for it to undergo fatty acid biosynthesis?

Answer 16

It must be shipped from the matrix of the mitochondrion to the cytosol via a transport system.

Question 17

What is a key steps in the biosynthesis of fatty acids?

Answer 17

Acetyl CoA -> Malonyl CoA

Enzyme: Acetyl CoA Carboxylase (ACC)

Reaction requires: biotin

Question 18

What is the most important enzyme in the regulation of lipogenesis? What activates this enzyme?

Answer 18

Acetyl-CoA Carboxylase; Citrate

Question 19

What this be?

Answer 19

Sphingomyelin

Question 20

What are two enzymes which regulate fatty acid oxidation?

Answer 20

Acetyl CoA Carboxylase

and

Carnitine Palmitoyl-Transferase

Question 21

How are free fatty acids carried?

Answer 21

BSA complexes

Question 22

What does Acyl-CoA inhibit?

Answer 22

The mitochondrial tricarboxylate transporter

Question 23

What feeds into the TCA cycle? What is produced?

Answer 23

Acetyl-CoA; ATP

Question 24

What are the clinical symptoms of Farber’s dz?

Answer 24

Question 25

What enzyme converts malate to pyruvate while producing NADPH?

Answer 25

The malic enzyme

Question 26

Describe the process of odd chain fatty acid oxidation.

Answer 26

Question 27

What are the clinical symptoms of Fabry's dz?

Answer 27

Question 28

What are the clinical symptoms of tay sachs?

Answer 28

Question 29

What is ketoacidosis?

Answer 29

Question 30

What the two substances that can be made from acetoacetate?

Answer 30

Acetone and D(-)-3-hydroxybutyrate

Question 31

Where are additional ezymes required in fatty acid synthesis found, which elongate and desaturate fatty acids?

Answer 31

The ER

Question 32

What are the four components of Coenzyme A?

Answer 32

The business end is circumscribed in red Remember: pantothetic acid

Question 33

What is the enzyme deficiency in tay sachs dz? accumulating lipid?

Answer 33

Hexosaminidase A; GM2 ganglioside

Question 34

What is required for most of the reductive steps involved in fatty acid biosynthesis?

Answer 34

NADPH

Question 35

What are the four consequences of sphingolipidoses (lipid storage dz)?

Answer 35

Question 36

Phosphatidic acid acts as a precursor to what?

Answer 36

Phospholipids and triglycerides

Question 37

How are ketone bodies broken down in order to feed into the TCA?

Answer 37

Question 38

What transporters are involved in transporting fatty acids into the mitochondrion?

Answer 38

Question 39

Describe the intermediates which go from cytosol -\> matrix of mitochondria? Matrix of mitochondria -\> cytosol?

Answer 39

Question 40

What is the enzyme deficiency in Niemann-Pick Dz? Accumulating lipid?

Answer 40

Sphingomyelinase; Sphingomyelin