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Medical Biochemistry > Lipid Metabolism > Flashcards

Lipid Metabolism Flashcards

1
Q

Where does FA synthesis occur?

A

Cytosol

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2
Q

What compounds are required for FA synthesis?

A

Acetyl CoA

NADPH

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3
Q

What produces Acetyl CoA in the mt matrix?

A

Pyruvate Dehydrogenase complex

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4
Q

How is Acetyl CoA transported into the cytosol?

A
  • Acetyl CoA + Oxaloacetate —> Citrate (citrate synthase)
  • Citrate is carried across membrane to
    Cytosol and converted back to Acetyl CoA and OAA
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5
Q

How is OAA returned to the mitochondria inFA Synthesis?

A
  • Converted to Malate via Malate dehydrogenase
  • Malate converted to pyruvate and CO2 via MAlic enzyme and NADP+
  • Pyruvate has a Carrier which allows transport across membrane
  • Pyruvate Carboxylase catalysis:
    Pyr + ATP + Biotin + CO2 —> OAA
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6
Q

What happens to Acetyl CoA once in the cytosol in FA synthesis?

A

Converted to Malonyl CoA via Acetyl CoA carboxylase

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7
Q

What is the coenzyme used in production of malonyl CoA?

A

Biotin

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8
Q

Why is consumption of Raw egg not recommended?

A

Raw egg contains Avidin, which can tie up biotin in other physiological processes.

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9
Q

What are the components of FA synthase?

A 
Acetyl Transferase (AT)
Malonyl Transferase(MT)
Keto Synthase (CE)
B-ketoacyl reductase (KR)
Dehydratase (DH)
Enology Reductase (ER)
Thioesterase (TE)
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10
Q

What is Phosphopantetheine? How is is attached to ACP Protein?

A

Prosthetic group which anchors the growing fattyacyl chain during FA synthesis

Attached covelantly

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11
Q

What is the role of Thioesterase I in Fatty Acid synthase?

A

Cleaves off the final product palmitate

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12
Q

What is Thioesterase II?

A

An independents protein in mammary glands which binds FA synthase causing premature release of short fatty acid chains

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13
Q

What are the first two rounds of FA synthesis?

A
  1. Butyryl-ACP formation

2. C-6 Formation

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14
Q

What is the #C and Bond numbers of palmitoleic acid?

A

16:2 (9,10)

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15
Q

What is the #C and Bond numbers of Oleic acid?

A

18:2 (9,10)

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16
Q

What is the important of thioesterase II activity for infants?

A

Smaller FA chains such as Capric, Lauric, and myristic acid are easy to digest compared to palmitic

17
Q

What are the primary products of FA oxidation?

A

NADH, FADH2, and Acetyl CoA

18
Q

Where does FA degradation occur?

A

Cytosol

19
Q

How is Fatty Acyl CoA transported into the mitochondria?

A

It is converted into fatty acyl Carnitine via Carnitine Acyltransferase

20
Q

Describe Carntineacyl-carnitine translocase disease?

A
  • Autosomal recessive mutation
  • Causes hypoketosis
  • Affects Liver, Cardiac muscle, skeletal muscle, arrhythmia
21
Q

What is the Net yield ATP from one Palmitate?

A

129 ATP

22
Q

How does oxidation of odd-number FAs differ?

A

Last product is Propionyl CoA

23
Q

How is propionyl CoA broken down?

A
  • Prop CoA carboxylase converts to D-methyl malonyl CoA
  • Converted to L-methyl malonyl CoA
  • Converted to Succinyl CoA via Methyl Malonyl CoA mutate and enters TCA
24
Q

What two compounds are necessary for breakdown of odd number FAs?

A

Biotin - Prosthetic group for PC carboxylase

Vitamin B12 - Coenzyme for MMC Mutase

25
Q

What is an example of alpha oxidation?

A

Digestion of Phytanic acid in dairy products and chlorophyll

26
Q

What enzyme hydroxylates phytanic acid?

A

A-hydroxylase

27
Q

What is important about omega-oxidation?

A
  • Significant for short chain fatty acids

- Involves oxidation of omega-methyl or adjacent methylene carbons

28
Q

What are the two allosteric regulators of FA synthesis/breakdown?

A
  1. Citrate - activates ACC

2. Malonyl CoA - Inhibits carnitine acyltransferase

29
Q

What s Refsum Disease?

A
  • Deficiency in alpha-hydroxylase enzyme
  • Accumulation of phytanic aid in plasma and nerve tissue
  • Leads to neurological damage
30
Q

Describe SIDS (Sudden Infant Death Syndrome)

A
  • Medium chain FA CoA dehydrogenase deficiency
  • Freq: 1: 10,000
  • Autosomal recessive
31
Q

Describe methylmalonic acidemia

A
  • Methylmalonyl CoA mutate sometimes missing
  • Sometimes B12 missing
  • Causes metabolic acidosis and retardation
32
Q

Describe the development of diabetic ketoacidosis

A

A. Increased glucagon/insulin ratio cause elevated cAMP in lover, resulting in decreased malonyl CoA

B. CPT-1 De-inhibition resulting in a activation Of FA oxidation

C. Mor Acetyl CoA produced leading to increased Ketone Bodies

Medical Biochemistry (26 decks)

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