Mitochondria and Peroxisomes Flashcards
Structure of a mitochondrion
It has a double membrane (proteins embedded into the the membranes):
> outer membrane
> inner membrane- folded to maximise the surface. The larger the surface, the more enzymes in the mitochondria.
Cristae- the folded membrane
Matrix- innermost lumen
Inter membrane space- space between outer and inner membranes.
Mitochondria Associate with Microtubules
Mitochondria is the Microtubules to move along them
Sometimes the mitochondria temporarily elongates along the Microtubules.
Mitochondrial plasticity
It’s structure is not static as it undergoes many different changes in shape
When does mitochondrial morphology changes?
Apoptosis
Ca2+ transfer- mitochondria stores a lot of Ca2+
Cell cycle
Nutrient starvation
Nutrient starvation induced mitochondrial tabulation
When starved, the mitochondria fuse with each other and elongate. So They cannot be enveloped by the ER and targeted for degradation in the autophagosomes through the autophagy pathway.
Production of mitochondrial proteins
Two separate genetic systems:
> mitochondria have their own genome- codes for a small no. of key proteins. The ribosomes in the mitochondria synthesise the proteins.
> vast majority of the proteins, that re specific to mitochondria, are encoded by the chromosomes in the nucleus. These proteins are synthesised in the cytoplasm and are then imported into the mitochondria.
Mitochondrial DNA
Circular
Inherited cytoplasmically from the mother
mtDNA is located in the matrix
The mitochondrial genetic code differs from the standard nuclear code
E.g. UGA is a stop codon in the nucleus but codes for tryptophan in mitochondria;
AGA and AGG usually code for arginine but are stop codons in the mitochondrial translation system.
Function of mitochondria
> breakdown of fatty acids to acetyl CoA
Decarboxylation of pyruvate (3C) to acetyl CoA ( 2C)
Citric Acid Cycle ( in the matrix)- a key precursor to the pathway that generate ATP
oxidative phosphorylation ( by enzymes in the inner membrane)- formation of ATP and water from ADP, Pi, reducing equivalents and Oxygen
thermogenesis- generation of heat
cell apoptosis- when a cell receives a signal for cell death, mitochondria release specific proteins ( cytochrome C) which interacts with other components in the cell to keep the apoptosis process going.
Localisaztion of mitochondria near sites of high ATP utilisation
Mitochondria are closely associated with muscle fibres as they deliver ATP to the machinery that helps catalyse muscle contraction.
Sperm tail- mitochondria elongated and wrapped around the flagellar axoneme- required for the movement of the sperm as a lot of ATP is required.
Beriberi
A neurological and cardiovascular disease that effects mitochondria as it is cause by deficiency of Vitamin B1. Vitamin B1 gives rise to thiamine prophosphate which is a prosthetic group of two mitochondrial enzymes, pyruvate dehydrogenase and alpha- ketoneglutarate dehydrogenase. Beriberi pateints have higher than normal conc. of these enzymes. These cannot be metabolised so they end up outside the cell.
Characteristics of Peroxisomes
Single membrane Crystalline material (contains the enzyme catalase) characterise the Peroxisomes.
Peroxisome biogenesis
- originate from the ER through a process called budding. Peroxisomal precursor vesicle formed that contains some of the membrane proteins that are typical of peroxisomes, e.g. specific proteins that catalyze protein import.
- peroxisomal proteins that are synthesised in the cytoplasm are imported into the peroxiome.
- they acquire additional membrane and through fission, can generate daughter peroxisomes. Growth by uptake of specific peroxisomal proteins and lipids from cytosol.
Structure and function of Peroxisomes
- They metabolise very long- chain fatty acids
- They perform steps in the synthesis of certain lipids e.g. cholesterol, plasmalogens, bile acids.
- Excess purine broken down ( AMP, GMP) to Uric acid
- Peroxisomes contains enzymes that use oxygen to oxidise substrates.
- Catalase breakdown hydrogen peroxide into water
- 0.2 - 1 micrometer in diameter
- No DNA or ribosomes
- Most peroxisomal proteins are encoded in the nucleus and synthesised in the cytoplasm.
- Some peroxisomal proteins are from the ER.