Mitochondria Flashcards

1
Q

Function of mitochondria

A
  • Biosynthetic pathways (aa; nucleotides, porphyrins; cholesterol, nitricoxide, glutathione)
  • Bioenergetic pathways ( aerobic oxidation of glucose; beta-oxidation of fatty acids)
  • Anti-oxidant defence
  • Redox status (NADH/NAD levels)
  • Cell Signaling (ROS; Ca2+)
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2
Q

Compartment of Mitochondria

A

Outer membrane
Inter membrane space
Inner membrane
Matrix

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3
Q

Outer membran

A

Contain large channels forming proteins (porin)

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4
Q

Inter membrane space

A

Contain several enzymes that use the ATP passing out of the matrix to phosphorylate other nucleotide
Proteins released during apoptosis

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5
Q

Inner membran

A

Folder into numerous crises
Contain proteins that carry out oxidative phosphorylation
Including the ETS and ATP synthase Enzym

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6
Q

Matrix

A

High concentration of mixtures of hundreds of enzymes including for oxidation

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7
Q

Special features of Mitochondria

A

Can change their shape, location and number to suit a cells needs

Can divide like a bacterium (fission)

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8
Q

The origins of Mitochondrial RNA and proteins

A

They produce their own proteins because they have their own DNA

Mitochondria Import most of their lipids, but converts some imported lipids to cardiolipin (four fatty acids)

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9
Q

Transport of proteins into mitochondria

A

Through transmembrane transport

The protein which would go in the mitochondria must take signal sequence to bind to the import receptor which open the channel
Then bind to the protein translocator and get in the matrix- signal secuence cut away and the protein became a conformation (mature)

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10
Q

How obtain cells their energy

A

Cells obtain most of their energy by a membrane-based mechanisms that use the energy provided by food or sunshine to generate ATP

  • from food=oxidative fosforilation
  • from light=Fotosynthese

Membrane based systems use the energy stored in an electrochemical proton gradient to synthesis ATP

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11
Q

Sugar and fats

A

Both are degraded to Acetyl-CoA in Mitochondria

Produce after the acetyl coa cycle NADH and FADH which go to ETS and used for ATP

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12
Q

aTP synthase

A

The movement of electrons is coupled to the pumping of protons across the inner mitochondrial membrane

Activated carriers generated during the citric acid cycle power the production of ATP

Mitochondria catalyze a major conversion of energy

NADH transfers its electrons to oxygen through three large respiratory enzyme complex

The proton gradient then drives ATP synthesis

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13
Q

Quinones

A

Carry electrons within the lipid bilayer

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14
Q

The evolution of energy generating system

A

First the cells make fermentation because their was no o2

  1. To make ph basic they hidroliz ATP
  2. Can move H away and don’t use energy
  3. Now they can control both

Some major events are believed to have occurred during the evolution of living organisms on earth. Oxygen entered earth atmosphere billions of years ago

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15
Q

The genetic system of Mitochondria

A

Mitochondria contain complete genetic systems

The organization of human mitochondrial genome shows that animal mitochondria contain the simplest genetic system known

mtDNA can be homoplasmic(one Typ mtDNA cells)
or heteroplasmic (Mutant and normal mtDNA together in one cell - in some diseases the mutant one growth)
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16
Q

mtDNA

A
The simplest Genetik system 
Contain a little number of gen as gDNA 
Copy hight
Not good repair system 
High mutation rate
17
Q

Common common clinical phenotypes of Mitochondrial DNA associated diseases

A
  • Significant clinical and genetic heterogeneity
  • Syndrom ich and non Syndromic
  • Often multi system diseases
  • Onset between birth and senescence
  • Common cause of chronic morbidity and more prevalent than previously through
18
Q

Common phenotypes in adults

A
PEO = progressive external ophthalmoplegia
KSS = kearns sayer Syndrom 
MERRF= myoclonic epilepsy with ragged red fibers 
MELAS = Mitochondrial encephalomyopathy, lactic acidosis and stroke
19
Q

Mitochondria contribut the aging

A

Oxidative damage driven mutations and accumulation of reactive species may be a factor in aging