PEROXISOMES
KUNAL
AND
RAGHAV
WHAT ARE PEROXISOMES
Peroxisomes are small, membrane-
enclosed organelles that contain
enzymes involved in a variety of
metabolic reactions, including several
aspects of energy metabolism.
FUNCTIONS AND FEATURES
Oxidases promote oxidation of lipids
( Beta-Oxidation of long chain fatty acids)
forming Acetyl CoA, H2O2 and Catalase
which liberate O2 from H2O2.
Peroxins help proteins with specific
signal sequence to peroxisome. Peroxisomal
membrane have specific proteins that transport
substances between Matrix and Cytosol.
FUNCTIONS AND FEATURES
 Matrix has 50+ Enzymes
that metabolize Lipids (long
chain fatty acids, cholesterol
etc), Purines , Amino Acids
and H2O2.
Clinical And Applications
H2O2 is responsible for oxidative stress. Catalase
present in peroxisomes is Anti-Oxidant and combat this
oxidative stress thereby protecting cells from toxic H2O2 .
Hence INREASED activity of CATALASE is an indicator of
Oxidative Stress.
Proliferation in peroxisome caused by
drugs act on nuclei receptors of cells aka
PPARs(Peroxisome Proliferation Activated Receptors).
They on activation binds with DNA and produce
alterations in mRNA production thereby altering
physiological effects of many tissues and organs.
ZELLWEGER SYNDROME occurs due to mutation
in gene coding for Peroxins or peroxisomal enzymes.
It is characterized by neurological impairment,
increased long chain fatty acids, abnormality in
synthesis of bile acids and reduced plasmogens.
The child dies within a year in this disorder.
PRIMARY HYPEROXALURIA occurs due to
defective metabolism of Glycine derived by
glycosylate
ASSOCIATED DISEASES
ASSOCIATED DISEASES
ADRENOLEUKODYSTROPHY occurs due
to insufficient oxidation of long chain fatty
acids by peroxisomes. It is an Autosomal
recessive disease that manifest with
degeneration of LIVER, KIDNEY and BRAIN.
It is also called as Brown-Schilder`s
disease. Symptoms may include difficulty
swallowing or understanding speech,
impaired hearing or vision, muscle spasms,
and seizures.
project science and technology.pptx

project science and technology.pptx

  • 1.
  • 2.
    WHAT ARE PEROXISOMES Peroxisomesare small, membrane- enclosed organelles that contain enzymes involved in a variety of metabolic reactions, including several aspects of energy metabolism.
  • 3.
    FUNCTIONS AND FEATURES Oxidasespromote oxidation of lipids ( Beta-Oxidation of long chain fatty acids) forming Acetyl CoA, H2O2 and Catalase which liberate O2 from H2O2. Peroxins help proteins with specific signal sequence to peroxisome. Peroxisomal membrane have specific proteins that transport substances between Matrix and Cytosol.
  • 4.
    FUNCTIONS AND FEATURES Matrix has 50+ Enzymes that metabolize Lipids (long chain fatty acids, cholesterol etc), Purines , Amino Acids and H2O2.
  • 5.
    Clinical And Applications H2O2is responsible for oxidative stress. Catalase present in peroxisomes is Anti-Oxidant and combat this oxidative stress thereby protecting cells from toxic H2O2 . Hence INREASED activity of CATALASE is an indicator of Oxidative Stress. Proliferation in peroxisome caused by drugs act on nuclei receptors of cells aka PPARs(Peroxisome Proliferation Activated Receptors). They on activation binds with DNA and produce alterations in mRNA production thereby altering physiological effects of many tissues and organs.
  • 6.
    ZELLWEGER SYNDROME occursdue to mutation in gene coding for Peroxins or peroxisomal enzymes. It is characterized by neurological impairment, increased long chain fatty acids, abnormality in synthesis of bile acids and reduced plasmogens. The child dies within a year in this disorder. PRIMARY HYPEROXALURIA occurs due to defective metabolism of Glycine derived by glycosylate ASSOCIATED DISEASES
  • 7.
    ASSOCIATED DISEASES ADRENOLEUKODYSTROPHY occursdue to insufficient oxidation of long chain fatty acids by peroxisomes. It is an Autosomal recessive disease that manifest with degeneration of LIVER, KIDNEY and BRAIN. It is also called as Brown-Schilder`s disease. Symptoms may include difficulty swallowing or understanding speech, impaired hearing or vision, muscle spasms, and seizures.