

At the end of this lecture the student will be
able to:
Define The cell organelles .
Compare between prokaryotic and
eukaryotic cells.
List the types of organelles.
Describe the Mitochondrial Inheritance .
Recognize to the Mitochondrial diseases .
Recognize to the clinical features of the
diseases in this lecture.

In cell biology: an
organelle
is
a specialized
subunit within a cell that has a specific
function, and it is usually separately enclosed
within it`s own lipid bilayer.
The name organelle comes from the idea that
these structures are to cells what an organ is
to the body (hence the name
organelle,
the
suffix
-elle
being a diminutive).
Organelles are identified by microscopy.
There are many types of organelles,
particularly in eukaryotic cells.
While prokaryotes do not possess organelles.

The prokaryotic cell
is
-
a simple cell.
-
It has no nucleus.
-
no membrane-bound organelles.
-
The genetic material of a prokaryotic cell is found
in a region of the cell known as the
nucleoid.
Bacteria are a fine example of prokaryotic cells
and divide by a process known as
binary fission
;
they duplicate their genetic material, divide in
half, and produce two identical daughter cells.

The eukaryotic cell:
is
-
much more complex.
-
It contains a nucleus, which functions as
the control center of the cell, directing
DNA replication, transcription, and cell
growth.
-
Eukaryotic organisms may be unicellular
or multicellular.
One of the key features of eukaryotic cells
is the presence of membrane-bound
organelles, each with it`s own duties
.

Eukaryotes
Prokaryotes
fungi, plants, animals
bacteria, archaea
Typical organisms
~ 10–100 µm
~ 1–5 µm
Typical size
true nucleus with
double membrane
nucleoid region; no
true nucleus
Type of nucleus
linear molecules
(chromosomes) with
histone proteins
circular (usually)
DNA

60S and 40S
50S and 30S
Ribosomes
highly structured by
endomembranes and a
cytoskeleton
very few structures
Cytoplasmic
structure
flagella and cilia containing
microtubules; lamellipodia
&filopodia containing actin
flagella made of flagellin
Cell movement
one to several thousand
(though some lack
mitochondria)
none
Mitochondria
in algae and plants
none
Chloroplasts
single cells, colonies, higher
multicellular organisms with
specialized cells
usually single cells
Organization
Mitosis (fission or budding)
Meiosis
Binary fission (simple division)
Cell division


Eukaryotic Organelles:
Ribosomes:
1)
ribosomes serve as the host organelle for
protein synthesis
.
2)
Eukaryotes have bound ribosomes, which are
attached to endoplasmic reticula and form
proteins that tend to be exported from the cell
or sent to the membrane.
3)
There are also free ribosomes, which exist
freely in the cytoplasm and produce proteins
that remain in the cytoplasm of the cell.
4)
Eukaryotic ribosomes are built in a structure
called the
nucleolus
.

Prokaryotic ribosomes
Eukaryotic ribosomes

Smooth endoplasmic reticulum:
This is a membrane-bound organelle involved
in lipid synthesis, detoxification, and
carbohydrate metabolism.
Liver cells contain a lot of
smooth endoplasmic
reticulum (SER) because they host a lot of
carbohydrate metabolism (glycolysis).
The liver contains much SER
for another
reason—it is the site of alcohol detoxification.
It is given the name "smooth" endoplasmic
reticulum
because there are no ribosomes on
its cytoplasmic surface
.


This membrane-bound organelle is
termed "rough"
because of the
presence of ribosomes on the
cytoplasmic surface of the cell.
The proteins produced by this
organelle are often secreted by the
cell and carried by vesicles to the
Golgi apparatus
for further
modification.


Proteins, lipids, and other macromolecules
are sent to the Golgi to be modified by the
addition of sugars and other molecules to
form
glycoproteins
.
The products are then sent in vesicles to
other parts of the cell, directed by the
particular changes made by the Golgi.
In other words
: storage & packaging of
materials that will be exported from the cell.



Nucleus
: This is the control center of the cell.
In eukaryotic cells, this is the storage site of
genetic material (DNA).
It is the site of replication, transcription, and
posttranscriptional modification of RNA. It also
contains the nucleolus, the site of ribosome
synthesis.
Vacuole
: This is a storage organelle that acts
as a vault.
Vacuoles are quite large in plant cells but
small in animal cells.

Nucleus
Vacuole

Cytoskeleton
:
The skeleton of cells consists of three
types of fibers that provide support, shape,
and mobility to cells:
microtubules
Microfilaments
intermediate filaments
1 – Microtubules:
are constructed from
tubulin
and have a
lead role in the separation of cells during cell
division.
Microtubules are also important components
of cilia and flagella, which are structures that
aid the movement of particles .

2- Microfilaments:
are constructed from
actin
,
play a big part in muscular
contraction.
3- Intermediate filaments:
are constructed from a class of
proteins called
keratins
and are
thought to function as
reinforcement for the shape and
position of organelles in the
cell.

.
.

Peroxisomes:
1-are single-membrane structures found
in all eukaryotic cells. They are small,
membrane-bound structures that use
molecular oxygen to oxidize organic
molecules.
2-These are organelles containing
enzymes that produce hydrogen peroxide
as a by-product while performing various
functions, such as breakdown of fatty
acids and detoxification of alcohol in the
liver.
3- also contain an enzyme that converts
the toxic hydrogen peroxide by-product of
these reactions into cell-friendly water.


are special types of vacuoles .
contain enzymes for use in the
hydrolytic breakdown of
macromolecules .
Three ways to enter a lysosome –
phagocytosis , autophagy, and
receptor-mediated endocytosis.


1.
Found in ALL eukaryotic cells (even in
plant cells)
2.
Site of aerobic respiration
sugars + O
2
- - > ATP + CO
2
+ H
2
O
So it called “powerhouse of the cell”
3.
Contain DNA which codes for
mitochondrial proteins, ribosomes,
etc.

4.
Divide by a process similar to binary fission
when cell divides.
5. Enclosed in a double membrane system.
1.
Inner Membrane forms the Cristae
(invaginations into interior region)
Site of energy generation (houses of
energy)
2.
Matrix is the soluble portion of the
mitochondria
A.
Site of carbon metabolism
B.
Location of mDNA
C.
Site of mitochondrial protein synthesis



Mammalian cells contain mitochondria which have
their own circular deoxyribonucleic acid (mtDNA).
Mitochondria are only passed on in the cytoplasm of
the egg so that a child inherits all mitochondria
from his mother.
There are some diseases that are some diseases that
are associated with mutations in mitochondrial DNA.
A woman with such a mutation will therefore pass it
on to all her children whilst a man with the same
mutation will not pass it on to any of his children.
There is often a wide range of severity of disease in
carriers of mitochondrial mutations. Mitochondrial
inheritance is characterized by:
1-Maternal transmission only.
2-Extreme variability in the same family.

The genetics of mtDNA differ from that of DNA in the
following unique properties:
1-The mitochondrial genome is maternally inherited:
The mother transmits her oocyte mtDNA to all of
her offspring & her daughters transmit their mtDNA
to the next generation. This is due to the fact that
during fertilization. The few mitochondria from the
sperm that enter the egg are rapidly eliminated
through unknown mechanism.
2-Mitochondria are polypoid:
Each human cell has hundreds of mitochondria
each containing 2-10 mtDNA molecules. At cell
division mitochondria and their genomes are
randomly distributed to daughter cells.

3-The mitochondrial genome has a much faster
evolution rate than the nuclear genome:
The average number of base pair difference
between two human mitochondrial genomes is
estimated to be from 9.5 to 66. This explained
by the fact that, although the mitochondrial
gamma-DNA polymerase may retain a
proofreading activity X certain types of repair
enzymes have been identified in mitochondrial
fractions.
Mitochondria lack an efficient DNA repair
system, based, for instance, on homologous
recombination or removal of pyrimidine
dimmers. In addition, the mitochondrial
genome lacks protective proteins like
histones, and is physically associated with the
inner mitochondrial membrane, where highly
mutagenic oxygen radicals are generated as
by-products of OXPHOS.

4- Normally, the mitochondrial genotype
of an individual is composed of a single
mtDNA species:
A condition known as homoplasmy.
However, the intrinsic propensity of
mtDNA to mutate randomly can
occasionally determine a transitory
condition known as heteroplasmy,
where the wild-type & the mutant
genomes co-exist intracellular.

Assorted myopathies and neuropathies observed
clinically in recent years:
neurologic disorders
ragged red fibers in muscle biopsies
weakness exacerbated by exercise
seizures
(muscle jumps)
dementia with early onsetmovement disorders
stroke-like symptoms
retinopathy
hearing loss
partial blindness
migraine headaches

Combined oxidative
phosphorylation
deficiency 5
.


.
CELL
STRUCTURE
LOCATIO
N
DESCRIPTIO
N
FUNCTIO
N
Cell Membrane
All cells
Plant - inside
cell wall
Animal -
outer layer;
cholesterol
Double layer
of
phospholipids
with proteins
Selectively
permeable
Support
Protection
Controls
movemen
t of
materials
in/out of
cell
Barrier
between
cell and
its
environm
ent
Maintains
homeosta
sis
Nucleus
All cells
except
prokaryotes
Large, oval
May contain
1 or more
nucleoli
Holds DNA
Controls
cell
activities
Contains
the
hereditary
material
of the cell
Nuclear membrane
All cells
except
prokaryotes
Surrounds
nucleus
Double
membrane
Selectively
permeable
Controls
movemen
t of
materials
in/out of
nucleus

.
Cytoplasm
All cells
Clear, thick,
jellylike
material
(cytosol)
Organelles
found inside cell
membrane
Contains the
cytoskeleton
fibers
Supports and
protects cell
organelles
Endoplasmic
reticulum (ER)
All cells
except
prokaryotes
Network of
tubes or
membranes
Smooth w/o
ribosomes
Rough with
embedded
ribosomes
Connects to
nuclear
envelope & cell
membrane
Carries
materials
through cell
Aids in
making
proteins
Ribosome
All cells
Small bodies
free or attached
to ER
Made of rRNA
& protein
Synthesizes
proteins
Mitochondrion
All cells
except
prokaryotes
Peanut shaped
Double
membrane
Outer
membrane
smooth
Inner membrane
folded into
cristae
Breaks down
sugar
(glucose)
molecules to
release
energy
Site of
aerobic
cellular
respiration

.
Lysosome
Plant -
uncommon
Animal -
common
Small and round
with a single
membrane
Breaks down
larger food
molecules
into smaller
molecules
Digests old
cell parts
nucleolus
All cells
except
prokaryotes
Found inside the
cell's nucleus
May have more
than one
Disappear
during cell
division
Make
ribosomes
Golgi Apparatus
All cells
except
prokaryotes
Stacks of
flattened sacs
Have a cis &
trans face
Modify
proteins
made by the
cells
Package &
export
proteins
Centrioles
Animal cells
Paired structures
near the nucleus
Made of a
cylinder of
microtubule
pairs
Separate
chromosome
pairs during
mitosis
Cytoskeleton
All cells
Made of
microtubules 7
microfilaments
Strengthen
cell &
maintains the
shape
Moves
organelles
within the
cell


.
Thank you