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BC2002: Principles Of Metabolic Pathways > Anabolic Pathways & Integration of Metabolism > Flashcards

Anabolic Pathways & Integration of Metabolism Flashcards

1
Q

How many genes do human mitochondria contain?

A

37

13 of which encode subunits of respiratory chain complexes

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2
Q

How are mitochondria inherited?

A

Maternally

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3
Q

Describe the Mitochondial Outer Membrane

A
  • Contain porin channels
  • Freely permeable to small molecules and ions
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4
Q

Describe the Mitochondrial Inner Membrane

A
  • Highly impermeable
  • Is highly convoluted - Cristae
  • Contains multiple copies of respiratory chain & ATP synthase
  • Multiple transporter proteins
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5
Q

Describe the Mitochondrial Matrix

A
  • Contains many catabolic enzyme systems e.g. Citric Acid Cycle, Beta-Oxidation, Pyruvate Dehydrogenase
  • Also contains DNA, Ribosomes, Ions, and Metabolites
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6
Q

What is Mitochondrial Fission?

A

Dividing of mitochondria

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7
Q

What is Mitochondrial Fusion?

A

Coming together of mitochondria

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8
Q

What is Mitochondrial Mitophagy?

A

Degredation of damaged or non-functional mitochondria

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9
Q

What are the major forms of mitochondrial disease?

A
  • Neurodegeneration
  • Cancer
  • Heart Disease
  • Diabetes
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10
Q

What are some therapies for maternally inherited mitochondrial disorders?

A

Mitochondrial Transplant

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11
Q

What happens when Reactive Oxygen Species (ROS) are overproduced in the mitochondria?

A

Cause oxidative stress leading to defective mitochondrial function –> defective cell function –> cell death in aging and diseases of ageing

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12
Q

What mitochondrial protein activates a cell signalling pathway that causes cell death?

A

Cytochrome C

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13
Q

How do mitochondrial defects contribute to cancer?

A
  • Disrupt OXPHOS
  • Promote “aerobic” glycolysis
  • Uncouple electron transport from ATP synthesis
  • Use mitochondrial metabolism for biosynthesis
  • Mutations in the genes encoding protein subunits of Complex I-IV can cause cancer
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14
Q

How do mitochondrial defects contribute to infection/immunity?

A
  • Pathogens can manipulate mitochondrial metabolism & OXPHOS to promote infection
  • When mitochondrial metabolism and dynamics are impaired, damaged mitochondria can cause inflammation
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15
Q

How do mitochondrial defects contribute to neurodegeneration?

A
  • Complex I activity is impaired & defects in ATP synthase
  • MPTP –> MPP+ inhibits Complex I
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16
Q

What is the role and importance of triacylglycerol & fatty acid synthesis?

A
17
Q

What are the starting biomolecules of triacylglycerols & fatty acids?

A

Acetyl-CoA & Malonyl-CoA

18
Q

Is fatty acid synthesis a reductive or oxidative process?

A

Reductive

19
Q

What form of reducing power is used in fatty acid synthesis - i.e. what biomolecule dontates electrons?

A

NADPH

20
Q

Is fatty acid synthesis a reversal of fatty acid oxidation?

A

No

It has totally distinct enzymes

21
Q

Where does fatty acid synthesis occur in the cell?

A

Cytosol

22
Q

How does the fatty acid synthesis process start and why is citrate exit from mitochondria important?

A
  • FA synthesis starts when cells have surplus energy - Citrate builds up in mitochondria
  • Citrate exit important because it reconverts into Acetyl-CoA & Oxaloacetate
23
Q

What enzyme is involved in reconverting Citrate back into Acetyl-CoA & Oxaloacetate?

A

ATP Citrate Lyase (ACL)

24
Q

What steps need to occur for citrate to exit mitochondria and convert to Acetyl-CoA?

A
  • Citrate builds up in mitochondria
  • Citrate exits via citrate transporter in INNER mitochondrial membrane and enters cytosol
  • Citrate reconverts back into Acetyl-CoA & Oxaloacetate
  • Acetyl-CoA now used for FA synthesis
25
Q

What is fatty acid synthase and what does it do?

A
  • Is an enzyme complex
  • Builds up fatty acid chain 2C at a time in a repetitive 4 step reductive sequence
26
Q

Give a general overview of the 4-step reductive process that synthesises the fatty acids

A
27
Q

Why are dietary fatty acids important?

Name 1 or 2

A

Because mammalian cells cannot synthesise double bonds between C10 & CH3 terminal

28
Q

What enzyme regulates fatty acid synthesis and how?

A

Acetyl-CoA Carboxylase (ACC)
* High Acetyl-CoA & Citrate = ACC ACTIVATED
* High AMP & Palmitoyl-CoA = ACC INHIBITED

29
Q

How does the regulation of fatty acid synthesis coordinate with fatty acid breakdown in beta-oxidation?

A
30
Q

Which mitochondrial protein activates a cell signalling pathway that causes cell death?

A

Cytochrome C

31
Q

Injection of MPTP results in the inhibition of which ONE of the following enzymes?

a) Glycogen phosphorylase
b) Pyruvate kinase
c) Hexokinase
d) Complex I (NADH dehydrogenase)
e) None of the above

A

d) Complex I (NADH dehydrogenase)

32
Q

Which ONE of the following biomolecules exits the mitochondria to convert to Oxaloacetate and Acetyl-CoA to commence fatty acid synthesis?

a) Asparate
b) Malate
c) Pyruvate
d) Fumarate
e) Citrate

A

e) Citrate

33
Q

Acetyl-CoA carboxylase is activated by which of the following biomolecules?

a) NAD
b) AMP
c) ADP
d) Citrate
e) None of the above

A

d) Citrate

34
Q

The fatty acid synthase complex is composed of which ONE of the following number of enzymes?

a) 5
b) 6
c) 12
d) 4
e) 20

A

b) 6

35
Q

The fatty acid synthase complex performs which ONE of the following sequences of enzymatic reactions?

a) Synthase, Phosphorylation, Hydratase, Carboxylase
b) Phosphorylation, Synthase, Dehydratase, Carboxylase
c) Synthase, Hydratase, Reductase, Reductase
d) Synthase, Reductase, Dehydratase, Reductase
e) Dehydratase, Dehydrogenase, Reductase, Synthase

A

d) Synthase, Reductase, Dehydratase, Reductase

36
Q

Insulin increases both fatty acid synthesis and glycogen synthesis by activating which ONE of the following two enzymes?

a) Carnitine acyltransferase-1 and glycogen phosphorylase
b) Acetyl-CoA carboxylase and glycogen synthase
c) Acetyl-CoA carboxylase and glycogen phosphorylase
d) ATP synthase and carnitine acyltransferase-1
e) None of the above

A

b) Acetyl-CoA carboxylase and glycogen synthase

BC2002: Principles Of Metabolic Pathways (6 decks)

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