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GI and Nutrition > 2.4.2 Metabolism III > Flashcards

2.4.2 Metabolism III Flashcards

1
Q

What are three of the essential fatty acids?

A

linoleic, linolenic, arachidonic acids

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2
Q

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

A

Providing carbons

Feeding the pentose phosphate pathway, which generates NADPH

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3
Q

What is the significance of ketogenesis?

A
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4
Q

What does reaction does insulin activate?

A

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

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5
Q

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

A

Ceramidase; Acyl-Sphingosine Ceramide

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6
Q

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

A

Beta-glucocerebrocidase; Glucosyl ceramide

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7
Q

What is multiple sclerosis? What is lost?

A

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

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8
Q

What are the clinical symptoms of niemann-pick dz?

A
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9
Q

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

A
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10
Q

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

A

alpha-galactosidase; globotricylceramide

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11
Q

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

A

Liver

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12
Q

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

A
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13
Q

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

A

Acyl carrier protein (ACP), which contains pantothenic acid

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14
Q

How is ceramide synthesized?

A
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15
Q

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

A

Glucagon and insulin

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16
Q

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

A

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

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17
Q

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

A

Acetyl CoA -> Malonyl CoA

Enzyme: Acetyl CoA Carboxylase (ACC)

Reaction requires: biotin

18
Q

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

A

Acetyl-CoA Carboxylase; Citrate

19
Q

What this be?

A

Sphingomyelin

20
Q

What are two enzymes which regulate fatty acid oxidation?

A

Acetyl CoA Carboxylase

and

Carnitine Palmitoyl-Transferase

21
Q

How are free fatty acids carried?

A

BSA complexes

22
Q

What does Acyl-CoA inhibit?

A

The mitochondrial tricarboxylate transporter

23
Q

What feeds into the TCA cycle? What is produced?

A

Acetyl-CoA; ATP

24
Q

What are the clinical symptoms of Farber’s dz?

A
25
Q

What enzyme converts malate to pyruvate while producing NADPH?

A

The malic enzyme

26
Q

Describe the process of odd chain fatty acid oxidation.

A
27
Q

What are the clinical symptoms of Fabry’s dz?

A
28
Q

What are the clinical symptoms of tay sachs?

A
29
Q

What is ketoacidosis?

A
30
Q

What the two substances that can be made from acetoacetate?

A

Acetone and D(-)-3-hydroxybutyrate

31
Q

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

A

The ER

32
Q

What are the four components of Coenzyme A?

A

The business end is circumscribed in red

Remember: pantothetic acid

33
Q

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

A

Hexosaminidase A; GM2 ganglioside

34
Q

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

A

NADPH

35
Q

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

A
36
Q

Phosphatidic acid acts as a precursor to what?

A

Phospholipids and triglycerides

37
Q

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

A
38
Q

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

A
39
Q

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

A
40
Q

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

A

Sphingomyelinase; Sphingomyelin

GI and Nutrition (57 decks)

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