Course: ADVANCE ZOOLOGY (ll)

Topic: peroxisome

Submitted by: Rukhsana Gulshan

BS.Ed (Hons) –V
Edu(s) – 2017-F-18

Submitted to: Ma’am Atya

FAZAIA COLLEGE OF EDUCATION FOR WOMEN

LAHORE CANTT

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Peroxisome:
Membrane-bound organelle occurring in the cytoplasm of eukaryotic cells. Peroxisomes play a key role
in the oxidation of specific biomolecules. They also contribute to the biosynthesis of membrane lipids
known as plasminogen . In plant cells, peroxisomes carry out additional functions, including the recycling
of carbon from phosphor glycolate during photorespiration. Specialized types of peroxisomes have been
identified in plants, among them the glyoxysome , which functions in the conversion of fatty acids to
carbohydrates.

Peroxisomes contain enzymes that oxidize certain molecules normally found in the cell, notably fatty
acids and amino acids. Those oxidation reactions produce hydrogen peroxide, which is the basis of the
name peroxisome. However, hydrogen peroxide is potentially toxic to the cell, because it has the ability
to react with many other molecules. Therefore, peroxisomes also contain enzymes such as catalase that
convert hydrogen peroxide to water and oxygen, thereby neutralizing the toxicity. In that way
peroxisomes provide a safe location for the oxidative metabolism of certain molecules.

History:
Peroxisomes were described in 1960 as part of the pioneering work of Christian René de Duve, who
developed cell fractionation techniques. De Duve’s method separated organelles on the basis of their
sedimentation and density properties, and peroxisomes are denser than other organelles. He later
coined the term peroxisome. De Duve shared the 1974 Nobel Prize for Physiology or Medicine
with Albert Claude and George Palade for that work.

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Plasmalogens:
Plasmologen are the primary ether lipids in humans (ether lipids contain one or more ether linkages,
distinguishing them from other lipids, which typically contain ester linkages). Specialized enzymes in
peroxisomes catalyze the synthesis of an ether phospholipid precursor. The precursor molecule
undergoes further synthesis in the endoplasmic reticulum, resulting in the production of plasmalogen.
Although the physiological role of plasmalogens is unclear, defects in their biosynthesis, which occur as a
result of peroxisomal disorders, are associated with severe developmental conditions, including
rhizomelic chondrodysplasia punctata (RCDP) and Zellweger syndrome.

Peroxisomal disorders:
They are caused by mutations in genes that are involved in peroxisome biogenesis or that encode the
enzymes and transporter proteins (which take up the enzymes from the cytoplasm) of the peroxisome.
Peroxisomal disorders are congenital disorders, and they range from relatively moderate to severe in
nature. The Zellweger spectrum, for example, includes Zellweger syndrome, neonatal
Adrenoleukodystrophy (NALD), and infantile Refsum disease. Zellweger syndrome is characterized by
complete absence or reduction in the number of peroxisomes. It is the most severe condition within the
Zellweger syndrome. Mutations giving rise to Zellweger syndrome cause copper, iron, and substances
called very long chain fatty acids to accumulate in the blood and in tissues, such as the liver, brain, and
kidneys. Infants with Zellweger syndrome are often born with facial deformity and intellectual disability;
some may have impaired vision and hearing and may experience severe gastrointestinal bleeding or liver
failure. Prognosis is poor: most infants with Zellweger syndrome do not live beyond one year.

Symptoms of NALD and infantile Refsum disease, by contrast, appear in late infancy or in childhood, and
patients may survive to early adulthood. Likewise, patients with RCDP may survive into childhood or, in
mild cases, early adulthood.

Functions of Peroxisomes:
Peroxisomes contain at least 50 different enzymes, which are involved in a variety of biochemical
pathways in different types of cells. Peroxisomes originally were defined as organelles that carry out
oxidation reactions leading to the production of hydrogen peroxide. Because hydrogen peroxide is
harmful to the cell, peroxisomes also contain the enzyme catalase, which decomposes hydrogen
peroxide either by converting it to water or by using it to oxidize another organic compound. A variety of
substrates are broken down by such oxidative reactions in peroxisomes, including uric acid, amino acids,
and fatty acids. The oxidation of fatty acids is a particularly important example, since it provides a major
source of metabolic energy. In animal cells, fatty acids are oxidized in both peroxisomes
and mitochondria, but in yeasts and plants fatty acid oxidation is restricted to peroxisomes.

Fatty acid oxidation in peroxisomes. The oxidation of a fatty acid is accompanied by the production of
hydrogen peroxide (H2O2) from oxygen. The hydrogen peroxide is decomposed by catalase, either by
conversion to water or by oxidation of another organic 

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Peroxisomes (micro bodies) were first described by a Swedish doctoral student, J. Rodin in 1954. They
were identified as organelles by the Belgian cytologist Christian de Duve in 1967, De Duve and co-
workers discovered that peroxisomes contain several oxidases involved in the production of hydrogen
peroxide (H2O2) as well as catalase involved in the decomposition of H 2O2 to oxygen and water. Due to
their role in peroxide metabolism, De Duve named them “peroxisomes”, replacing the formerly used
morphological term “micro bodies”. Later, it was described that firefly luciferase is targeted to
peroxisomes in mammalian cells, allowing the discovery of the import targeting signal for peroxisomes…

Structure:
Peroxisomes are small (0.1-1 µm diameter) subcellular compartments (organelles) with a fine, granular
matrix and surrounded by a single bio membrane which are located in the cytoplasm of a
cell. Compartmentalization creates an optimized environment to promote various metabolic reactions
within peroxisomes required to sustain cellular functions and viability of the organism.

The number, size and protein composition of peroxisomes are variable and depend on cell type and
environmental conditions. For example, in baker's yeast (S. cerevisiae), it has been observed that, with
good glucose supply, only a few, small peroxisomes are present. In contrast, when the yeasts were
supplied with long-chain fatty acids as sole carbon source up to 20 to 25 large peroxisomes can formed.

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Metabolic functions:
A major function of the peroxisome is the breakdown of very long chain fatty acids through beta
oxidation. In animal cells, the long fatty acids are converted to medium chain fatty acids, which are
subsequently shuttled to mitochondria where they eventually are broken down to carbon dioxide and
water. In yeast and plant cells, this process is carried out exclusively in peroxisomes.

The first reactions in the formation of plasmalogen in animal cells also occur in peroxisomes.
Plasmalogen is the most abundant phospholipid in myelin. Deficiency of plasmalogens causes profound
abnormalities in the myelination of nerve cells, which is one reason why many peroxisomal
disorders affect the nervous system. Peroxisomes also play a role in the production of bile acids
important for the absorption of fats and fat-soluble vitamins, such as vitamins A and K. Skin disorders
are features of genetic disorders affecting peroxisome function as a result

The specific metabolic pathways that occur exclusively in mammalian peroxisomes are: ]

 α-oxidation of phytanic acid

 β-oxidation of very long-chain and polyunsaturated fatty acids

 biosynthesis of plasmalogens

 conjugation of cholic acid as part of bile acid synthesis

Peroxisomes contain oxidative enzymes, such as D-amino acid oxidase and uric acid oxidase However

the last enzyme is absent in humans, explaining the disease known as gout, caused by the accumulation
of uric acid. Certain enzymes within the peroxisome, by using molecular oxygen, remove hydrogen
atoms from specific organic substrates (labeled as R), in an oxidative reaction, producing hydrogen
peroxide (H2O2, itself toxic):

Catalase, another peroxisomal enzyme, uses this H 2O2 to oxidize other substrates
,including phenols, formic acid, formaldehyde, and alcohol, by means of the peroxidation reaction, thus
eliminating the poisonous hydrogen peroxide in the process.

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This reaction is important in liver and kidney cells, where the peroxisomes detoxify various toxic
substances that enter the blood. About 25% of the ethanol that humans consume by drinking alcoholic
beverages is oxidized to acetaldehyde in this way. When excess H2O2 accumulates in the cell, catalase
converts it to H2O.

There is evidence now that those reactive oxygen species including peroxisomal H 2O2 are also important
signaling molecules in plants and animals and contribute to healthy ageing and age-related disorders in
humans.

The peroxisome of plant cells is polarized when fighting fungal penetration. Infection causes
a glucosinolate molecule to play an antifungal role to be made and delivered to the outside of the cell
through the action of the peroxisomal proteins (PEN2 and PEN3).

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References:
M.W. Gray, in Encyclopedia of Genetics, 2001

https://bscb.org/learning-resources/softcell-elearning/peroxisome/

https://rarediseases.info.nih.gov/diseases/7917/zellweger-syndrome

https://scienceaid.net/the_Structure_and_Function_of_Peroxisomes

https://biologydictionary.net/peroxisome/

https://www.ncbi.nlm.nih.gov/books/NBK9930/

https://www.sciencedirect.com/topics/biochemistry-genetics-and-molecular-biology/peroxisome

https://en.wikipedia.org/wiki/Peroxisome

https://www.ncbi.nlm.nih.gov/books/NBK9930/