Mitochondrion - structure,

function & transmembrane

traffic
Cell Biology
CHAPT 14

The mitochondrion
Double membrane.
n Typically 0.5 to 1 m in diameter & up to
10 m long.
n Many per cell (1,000s).
n Typically 22% of cell volume in total.
n Contains DNA.
n Mobile and plastic (can change shape).
n Often associated with microtubules control of orientation/movement within
cells.
n Form long, moving chains in some cells.
n

Structure of the mitochondrion

Compartment

% total
mitochondrial
protein

Outer membrane

Inner membrane

21

Intermembrane space
Matrix

6
67

Outer membrane
Contains many copies of a transport
protein, porin, which forms large aqueous
pores through lipid bilayer.
n Membrane therefore permeable to
molecules of 5 kD or less.
n Also contains enzymes for conversion of
lipids prior to metabolism in the matrix.
n As inner membrane only selectively
permeable, composition of intermembrane
space with respect to molecules up to 10
kD is similar to cytosol and different to
matrix.
n

Inner membrane
Highly convoluted, forming cristae.
n Surface area 5 times that of outer
membrane.
n Combined area of mitochondrial
membranes is 1/3 of total
membrane in cell.
n Morphology of cristae differs in cells
of different functional type.
n Lipid bilayer is impermeable to H+
(and other ions generally).
n Equipped with carrier proteins that
mediate the entry & exit of essential
metabolites.
n

Overview of mitochondrial
function

Large amounts of acetyl CoA produced in

matrix space from pyruvate & fatty acids.
n Citric acid cycle oxidises acetyl CoA within
the matrix, generating NADH & FADH2
which are passed the respiratory chain on
the inner membrane.
n A chemiosmotic process located on the
inner membrane converts oxidation energy
into ATP.
n Electrochemical gradient is also used to
accumulate metabolites into matrix space;
2+
n and also to accumulate Ca , keeping the
concentration in the cytosol low.
n

A summary of energy-generating metabolism in

mitochondria

Copyright 2002 Garland Science Publishing, Inc.

Electron transport chain

Copyright 2002 Garland Science Publishing, Inc.

Biogenesis of mitochondria
n Minority of proteins encoded on
mitochondrial DNA.
n Majority of proteins encoded on nuclear
DNA & imported into mitochondria.
n Protein synthesis in mitochondria and
cytosol blocked by different antibiotics.
n Mitochondria always produced by growth
and division - never de novo.
n Division process reminiscent of bacterial
binary fission.
n Mitochondria divide throughout interphase.
n No. of mitochondria doubles during cell
cycle.

Comparison of protein transport through

mitochondrial & ER membranes

" Differences

Mitochondrion has double membrane.

n Import to ER by vectorial discharge
beginning with incomplete protein;
n mitochondrial import occurs after
translation complete.
n

" Similarities
Precursor protein contains signal sequence.
n Signal sequence directs protein to correct
destination.
n In many cases, signal sequence cleaved on
completion of transport.
n

MITOCHONDRIAL DYNAMICS

Transport through 2 membranes

Mitochondrial import via signal sequences

No Mitochondrial Export

Signal is structural, amphiphatic helix

Porins, Mito Outer Membrane

Post translational membrane translocation

ATP
Signal peptidase
Chaperonins, Heat Shock Proteins

MITOCHONDRIAL GENOMES

MITOCHONDRIAL GENOME

RELAXED CODON USAGE

Assembled on cytoskeletal filaments

Dynamic