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Metabolic Pathways > Mitochondria > Flashcards

Mitochondria Flashcards

1
Q

How many genes does the mitochondria contain?

A

37 genes, 13 of which encode subunits of the respiratory chain complexes
Nuclear genes encode most of the mitochondrial proteins.

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2
Q

How are mitochondrial genes inherited?

A

Maternally

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3
Q

How many membranes do mitochondria have?

A

2 membranes - Outer,(OMM) very permeable. Inner,(IMM) impermeable.

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4
Q

What does the IMM contain?

A

Highly convoluted with invaginations - cristae containing multiple copies of respiratory chain and ATP synthase.

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5
Q

There are multiple transporter proteins in the IMM, give an example?

A

Carnitine.

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6
Q

What does the matrix of the mitochondria contain.

A

Many catabolic enzyme systems:List on diagram (examples: DNA, ribosomes, ions, metabolites , citric acid cycle, beta oxidation, pyruvate dehydrogenase and for amino acid oxidation.

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7
Q

How are mitochondria highly dynamic? Why is this done?

A

They are constantly dividing by fission - to get rid of damaged mitochondria.

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8
Q

When does a lot of fission happen?

A

During anabolism

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9
Q

What is it called when mitochondria come together?

A

fusion

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10
Q

What is it called when mitochondria are being degraded when damaged or non-functional?

A

Mitophagy, proteins are also degraded by this.

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11
Q

What can differ between mitochondria cell types?

A

The cell need for high energy demand etc.

Number of cristae can change depending on the metabolic need.

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12
Q

What type of mutations in genes encoding for mitochondrial proteins can cause disease?

A

Inherited optic neuropathies.

- degeneration of retinal cells (loss of vision)

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13
Q

What causes Inherited optic neuropathies?

A

Missense mutation in mitochondria genes encoding subunits of complex 1.
Maternal (primarily)and nuclear inherited.

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14
Q

What is Leigh disease?

A

This is a neurological and psychomotor dysfunction - due to mutations in both mitochondrial and nuclear genes that encodes for protein complexes of mt chain complex IV

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15
Q

What is MELAS?

A

Mitochondrial encephalopathy with lactic acidosis and stroke like episdoes.

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16
Q

What is MERFF?

A

Myoclonic epilepsy with red ragged fibres.

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17
Q

MELAS is the most common cause of?

A

Mutations in mitochondrial gene encoding tRNA necessary for synthesis of mitochondrial proteins and this is also cause of MERFF

18
Q

What do diseases MERFF and MELAS have?

A

Defective mitochondrial protein synthesis.

19
Q

What is visible in the mitochondria of MERFF patients?

A

Skeletal muscle has abnormal shaped mitochondria with crystalline deposits.

20
Q

When was the first mitochondrial transplant ?

A

In 2015 UK approval of embryonic mitochondrial transfer to treat chronic maternally inherited mitochondrial diseases (3 person baby)

21
Q

What does the mitochondrial transplant consist of?

A
  • chromosomes removed from mutated m mother
  • put into healthy donor egg
  • egg fertilised
  • normal embryo develops, mother chromosome, male chromosome, donor mitochondria.
22
Q

What are ROS?

A

These are produced by mitochondria - reactive oxygen species.

23
Q

Which levels of ROS can be beneficial?

A

Low levels of ROS can be beneficial and cleared by effective antioxidant responses.

24
Q

What can happen if the electron transport is not correctly coupled to ATP production and O2 consumption?

A

ROS can be over produced.

25
Q

Why are ROS levels so important?

A

As it is very interactive, ot can bind to proteins/enzyme lipid and nucleic acids causing them to malfunction.

26
Q

What can high ROS levels lead to?

A

Oxidative stress leading to defective mitochondrial function - defective cell function or cell death in aging and diseases of ageing such as heart disease, neurodegeneration, type 2 diabetes etc.

27
Q

What is the role of cytochrome C in apoptosis?

A

Cytochrome C is released from mitochondria and activated a signalling pathway that causes cell death.

28
Q

What does apoptosis activate?

A

Apoptosome which further activates the caspase cascade.

29
Q

What metabolic systems are present in many cancer cell types?

A

Aerobic glycolysis and reduced OXPHOS.

Pyruvate to lactate they like to use this - anaerobic glycolysis.

30
Q

What do many oncogenes cause?

A

Metabolic requiring that disrupt OXPHOS, promoting aerobic glycolysis and uncouple the electron transport from ATP synthesis.

31
Q

What do cancer use mitochondrial metabolism for?

A

For biosynthesis.

32
Q

Can mutations in the genes encoding protein subunits cause cancer?

A

Yes of complexes I - IV

33
Q

What does cancer change in mitochondrial morphology?

A

Fission/fusion and mitophagy.

Increased rates of mitochondrial fission can promote more rapid cell division.

34
Q

What can some pathogens do to the immune system?

A

They can manipulate mitochondrial metabolism and OXPHOS to promote infection.

35
Q

Why is metabolic/mitochondrial bioenergy response important in the immune system?

A

Important in producing an effective immune response and reducing inflammation.

36
Q

What can happen when mitochondrial metabolism and dynamics are impaired?

A

Damaged mitochondria can be dangerous and cause inflammation.
Age related?

37
Q

More mitochondrial DNA in blood leading to ?

A

Death by Sars CoV 2

38
Q

What is Parkinsons?

A

This is a slowly progressive degenerative disorder of the central nervous system that impairs the person’s motor skills and other functions.

39
Q

Which complex is affected along with defects in ATP synthase in neurodegeneration?

A

Complex 1

40
Q

What are risk genes?

A

Loss of pink 1 function which causes parkinson’s has an effect in the balance between fission and fusion and a number of signalling pathways.

41
Q

What is Parkinson’s disease & MPTP?

A

A mitochondrial toxin

42
Q

What was the wrong chemistry involved in the case of the frozen addicts?

A

MPTP was accidentally produced during the manufacturing of MPPP.

Metabolic Pathways (13 decks)

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