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Concept 6.

4: The endomembrane system regulates


protein traffic and performs metabolic functions

The endomembrane system consists of


Nuclear envelope
Endoplasmic reticulum

connected

Golgi apparatus
Lysosomes
Vacuoles

Communicate
using vesicle
transport

Plasma membrane

The Endoplasmic Reticulum: Biosynthetic


Factory
The endoplasmic
reticulum (ER)
accounts for more
than half of the total
membrane in many
eukaryotic cells
The ER membrane
is continuous with
the nuclear
envelope

The Endoplasmic Reticulum: Biosynthetic


Factory
There are two
distinct regions of
ER
Smooth ER, which
lacks ribosomes
Rough ER, whose
surface is studded
with ribosomes
Cells usually have
some of both types,
but is often
predominate

Dynamics of the endoplasmic reticulum in a


live cell

Functions of Smooth ER
Synthesizes lipids
Metabolizes
carbohydrates
Detoxifies drugs
and poisons,
including alcohol
Stores calcium
ions

Microscopyu.com

The Golgi Apparatus: Shipping and Receiving Center

Functions of Rough ER
Has bound
ribosomes, which
synthesize proteins
and glycoproteins
(proteins covalently
bonded to
carbohydrates)

Made up of flattened
membranous sacs called cis
and trans cisternae
Not physically connected to
other membrane systems,
but receives and sends
vesicles to other organelles
Functions:

Distributes transport
vesicles, secretory
proteins surrounded
by membranes

Modifies proteins
produced by the RER

Is a membrane
factory for the cell

Sorts and packages


proteins into transport
vesicles

Manufactures certain
macromolecules

Plasma cell

Cisternal Maturation
Model: Cisternae move
from cis to trans

Video: ER to Golgi Traffic

2014 Pearson Education, Inc.

Video: Secretion from the Golgi

Lysosomes: Digestive Compartments


A lysosome is a
membranous sac of
hydrolytic enzymes that
can digest macromolecules

2 major functions of lysosomes:

Lysosomal enzymes work


best in the acidic
environment inside the
lysosome. This protect the
cell against accidental
digestion
Hydrolytic enzymes and
lysosomal membranes are
made by rough ER and
then transferred to the
Golgi apparatus for further
processing before forming
lysosomes

Tay Sachs Disease is caused by defects in lysosomal proteins


Over 50 clinically
recognized
diseases result
from lysosomal
defects

A baby born with Tay-Sachs develops normally in


the first 3 to 6 months of life. During the next
months or even years the baby will
progressively lose the ability to see, hear, and
move. By the age of 2, the child may have seizures
and become completely disabled. Death usually
occurs by the time the child is 5 years old.

Vacuoles: Maintenance Compartments


Vacuoles are
large vesicles
derived from
the ER and
Golgi apparatus
Vacuoles
perform a
variety of
functions in
different kinds
of cells

Food vacuoles are


formed by phagocytosis
Contractile vacuoles,
found in many
freshwater protists,
pump excess water out
of cells
Central vacuoles,
found in many mature
plant cells, hold organic
compounds and water

The endomembrane system:


A series of physically or functionally
connected membrane organelles
Nuclear envelope, endoplasmic reticulum,
golgi apparatus, lysosomes, vacuoles and
plasma membrane. Know their relationship and
specific function

Concept 6.5: Mitochondria and


chloroplasts change energy from one
form to another
Mitochondria are the
sites of cellular respiration,
a metabolic process that
uses oxygen to
generate ATP

Involved in protein synthesis, modification,


transport, storage and degradation

Chloroplasts, found in
plants and algae, are the
sites of photosynthesis

Perform a variety of other cell-type specific


functions

Peroxisomes are oxidative


organelles

The Evolutionary Origins of Mitochondria


and Chloroplasts
Mitochondria and chloroplasts
have similarities with bacteria
Enveloped by a double
membrane

The engulfed cell formed a


relationship with the host
cell, becoming an
endosymbiont

Contain free ribosomes and


circular DNA molecules
Grow and reproduce
somewhat independently
in cells
These and other similarities led
to the endosymbiont theory

The endosymbiont theory


suggests that an early
ancestor of eukaryotes
engulfed an oxygen-using
nonphotosynthetic
prokaryotic cell

The endosymbionts
evolved into mitochondria

DNA staining of a cell


(orange)

At least one of these cells


may have then taken up a
photosynthetic prokaryote,
which evolved into a
chloroplast

Mitochondria: Chemical Energy


Conversion

Mitochondria number varies with a cells energy needs.

In nearly all eukaryotic cells


Smooth outer membrane
Inner membrane folded into
cristae
Cristae present a large
surface area for ATP
synthesis

cardiac muscle

The inner membrane creates


the intermembrane space
and mitochondrial matrix
Some metabolic steps of
cellular respiration occur in
the matrix

Fig 14-6.

Ribosomes in matrix

Sperm
axoneme

chondrocyte

2014 Pearson Education, Inc.

Over 40 human
diseases or
disorders are
caused by
alteration to
mitochondrial
proteins.

Cristae in a liver hepatocyte..

..and the
cricothyroid muscle
of a bat

2014 Pearson Education, Inc.

'The Cell, 2nd Ed.' by Don W.


Fawcett M.D

http://www.umdf.org/mitodisease/

In September 2012, Britains


Human Fertilization and
Embryology Authority, which
deals with such matters,
launched a public
consultation to discuss the
ethics of creating threeparent offspring of the sort Dr
Mitalipov proposes. This
consultation runs till
December 7th and the results
will be given to the
government in the spring.

Mitochondrial theory of aging: Mitochondrial DNA is


unprotected by conventional DNA protection and repair
processes, yet is associated with a system
producing an abundance of
free radicals

http://www.economist.com/news/science-andtechnology/21565138-technique-intended-eliminatemitochondrial-diseases-would-result-people

Chloroplasts: Capture of Light Energy

Chloroplast structure includes


Outer and Inner membranes
Internal Thylakoids, membranous sacs, stacked to
form a granum
Stroma, the internal fluid
The chloroplast is one of a group of plant organelles,
called plastids. Other members store starch or pigments.
Ribosomes

Stroma
Inner
and outer
membranes

Chloroplasts contain the green pigment chlorophyll, as well as enzymes


and other molecules that function in photosynthesis

Granum

Chloroplasts are found in leaves and other green organs of plants and in
algae
Almost all free oxygen (O2) and almost all food on the planet has been
created by photosynthesis

Thylakoid

DNA
Intermembrane space

1 m

(a) Diagram and TEM of chloroplast


2014 Pearson Education, Inc.

Peroxisomes: Oxidation
Peroxisomes are specialized
metabolic compartments
bounded by a single
membrane
Peroxisomes scavenge
reactive oxygen, produce
hydrogen peroxide and
convert it to water
Peroxisomes perform
reactions with many different
functions
How peroxisomes are related
to other organelles is still
unknown
2014 Pearson Education, Inc.

Peroxisomal disorders
normal
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.

Zellweger syndrome
Infantile Refsum disease
Neonatal adrenoleukodystrophy
Pipecolic acidemia
Acatalasia
Hyperoxaluria type 1
Acyl-CoA oxidase deficiency
D-bifunctional protein deficiency
Dihydroxyacetonephosphate
acyltransferase deficiency
X-linked adrenoleukodystrophy
-Methylacyl-CoA racemase
deficiency
RCDP Type 2
RCDP Type 3
Adult Refsum disease
Mulibrey nanism

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