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Questions and Answers
What happens to peroxisomes as more proteins are imported into them?
What happens to peroxisomes as more proteins are imported into them?
- They become non-functional.
- They undergo fission. (correct)
- They shrink in size.
- They alter their membrane composition.
Which of the following is true about Zellweger syndrome?
Which of the following is true about Zellweger syndrome?
- It is an autosomal recessive disorder. (correct)
- Patients typically have normal peroxisome function.
- It can be treated effectively with diet alone.
- It is caused by dominant mutations in peroxin genes.
What compounds accumulate in individuals with impaired peroxisome function?
What compounds accumulate in individuals with impaired peroxisome function?
- Saturated fatty acids
- Short-chain fatty acids
- Triglycerides
- Very long chain fatty acids and branched chain fatty acids (correct)
Which proteins are required for the normal assembly of peroxisomes?
Which proteins are required for the normal assembly of peroxisomes?
Where do the receptor proteins involved in peroxisomal function primarily interact?
Where do the receptor proteins involved in peroxisomal function primarily interact?
What is one of the consequences of mutations in peroxin genes?
What is one of the consequences of mutations in peroxin genes?
Which mutation pattern is most common among patients with Zellweger syndrome?
Which mutation pattern is most common among patients with Zellweger syndrome?
What is the initial cellular response to the accumulation of fatty acids due to peroxisome impairment?
What is the initial cellular response to the accumulation of fatty acids due to peroxisome impairment?
What is a characteristic facial abnormality associated with peroxisomal disorders?
What is a characteristic facial abnormality associated with peroxisomal disorders?
Which condition is related to impaired neuronal development and function as a result of peroxisomal disorders?
Which condition is related to impaired neuronal development and function as a result of peroxisomal disorders?
What is a consequence of the accumulation of lipids in peroxisomal disorders?
What is a consequence of the accumulation of lipids in peroxisomal disorders?
Which statement is true about the developmental effects of peroxisomal disorders?
Which statement is true about the developmental effects of peroxisomal disorders?
What must occur before fatty acids can be oxidized in the mitochondria?
What must occur before fatty acids can be oxidized in the mitochondria?
Which statement about fatty acid oxidation is correct?
Which statement about fatty acid oxidation is correct?
What neurological issue is commonly reported in patients with Zellweger syndrome?
What neurological issue is commonly reported in patients with Zellweger syndrome?
What physiological characteristic is commonly observed in children affected by peroxisomal disorders?
What physiological characteristic is commonly observed in children affected by peroxisomal disorders?
What significant role do peroxisomes play in cellular metabolism?
What significant role do peroxisomes play in cellular metabolism?
How are peroxisomes different from lysosomes in terms of origin?
How are peroxisomes different from lysosomes in terms of origin?
Which process is primarily associated with the detoxification function of peroxisomes?
Which process is primarily associated with the detoxification function of peroxisomes?
What distinguishes peroxisomes from mitochondria in terms of genetic material?
What distinguishes peroxisomes from mitochondria in terms of genetic material?
What was the original term replaced by 'peroxisomes' as per De Duve's naming?
What was the original term replaced by 'peroxisomes' as per De Duve's naming?
What is the main mechanism involved in the importation of proteins into peroxisomes?
What is the main mechanism involved in the importation of proteins into peroxisomes?
Which function is NOT associated with peroxisomes?
Which function is NOT associated with peroxisomes?
What cellular process is triggered by the discovery of firefly luciferase's targeting to peroxisomes?
What cellular process is triggered by the discovery of firefly luciferase's targeting to peroxisomes?
What is produced during each round of fatty acid oxidation?
What is produced during each round of fatty acid oxidation?
Which enzyme is responsible for catalyzing the reverse reaction in beta oxidation when the R-group is a hydrogen?
Which enzyme is responsible for catalyzing the reverse reaction in beta oxidation when the R-group is a hydrogen?
Which of the following statements about beta oxidation reactions is true?
Which of the following statements about beta oxidation reactions is true?
Where is Acetyl-CoA released during fatty acid metabolism and where is it utilized?
Where is Acetyl-CoA released during fatty acid metabolism and where is it utilized?
What is the significance of an enzyme's role in the medium form concerning Sudden Infant Death Syndrome (SIDS)?
What is the significance of an enzyme's role in the medium form concerning Sudden Infant Death Syndrome (SIDS)?
What role do free fatty acids (FFAs) play during normal starvation and sepsis?
What role do free fatty acids (FFAs) play during normal starvation and sepsis?
Which transcription factor is primarily responsible for the induction of genes involved in β-oxidation of fatty acids?
Which transcription factor is primarily responsible for the induction of genes involved in β-oxidation of fatty acids?
What happens to peroxisomal PPAR-α levels during sepsis?
What happens to peroxisomal PPAR-α levels during sepsis?
Which process in the peroxisomes is essential for lipid metabolism?
Which process in the peroxisomes is essential for lipid metabolism?
What is a significant consequence of the accumulation of free fatty acids in organs during sepsis?
What is a significant consequence of the accumulation of free fatty acids in organs during sepsis?
How are FFAs affected by insulin during sepsis?
How are FFAs affected by insulin during sepsis?
What is the primary function of catalase in the peroxisomes?
What is the primary function of catalase in the peroxisomes?
What can be inferred about the relationship between adipose tissue lipolysis and energy availability during sepsis?
What can be inferred about the relationship between adipose tissue lipolysis and energy availability during sepsis?
Flashcards
Lipolysis in Fasting
Lipolysis in Fasting
Breakdown of fats (lipolysis) is increased during fasting to provide energy.
Insulin's role in Lipolysis
Insulin's role in Lipolysis
Insulin normally inhibits lipolysis (fat breakdown), while in sepsis, insulin resistance overrides this inhibition, allowing lipolysis.
FFA breakdown in sepsis
FFA breakdown in sepsis
Breakdown of fatty acids (Beta-oxidation) is impaired during sepsis, leading to accumulation of FFAs in organs (and blood).
PPAR-α role in fasting
PPAR-α role in fasting
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PPAR-α role in sepsis
PPAR-α role in sepsis
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Peroxisome function
Peroxisome function
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Sepsis energy crisis
Sepsis energy crisis
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Beta-oxidation in different cells
Beta-oxidation in different cells
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Peroxisomes
Peroxisomes
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Hydrogen Peroxide Detoxification
Hydrogen Peroxide Detoxification
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Peroxisome Import
Peroxisome Import
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Targeting Signal
Targeting Signal
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Liver Peroxisomes
Liver Peroxisomes
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Phospholipid Production
Phospholipid Production
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Microbodies
Microbodies
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Lysosomes
Lysosomes
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Zellweger syndrome
Zellweger syndrome
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Peroxins
Peroxins
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PEX genes
PEX genes
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VLCFA
VLCFA
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BCFA
BCFA
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Peroxisome fission
Peroxisome fission
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Peroxisome assembly.
Peroxisome assembly.
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Fatty Acid Oxidation - Steps
Fatty Acid Oxidation - Steps
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Beta Oxidation Reaction 1
Beta Oxidation Reaction 1
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Beta Oxidation Reaction 2
Beta Oxidation Reaction 2
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Beta Oxidation Reaction 3
Beta Oxidation Reaction 3
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Beta Oxidation Reaction 4
Beta Oxidation Reaction 4
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Peroxisomal disorders
Peroxisomal disorders
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Characteristic facial abnormalities
Characteristic facial abnormalities
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Hypotonia
Hypotonia
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Hypomyelination
Hypomyelination
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Fatty Acid Oxidation
Fatty Acid Oxidation
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Beta Oxidation
Beta Oxidation
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Fatty Acid Activation
Fatty Acid Activation
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Study Notes
Lipid Utilization During Fasting
- Hormonal pathways, substrates, organelles, key enzymes, and signaling molecules all promote lipid utilization during fasting.
Adipose Tissue Lipolysis
- Adipose tissue lipolysis is a crucial process during fasting.
- Key enzymes like ATGL and HSL break down triglycerides (TGs) into free fatty acids (FFAs).
- Glycerol is another byproduct of this process.
- Hormones like epinephrine and glucagon stimulate lipolysis.
- FFAs are transported to other tissues for energy production.
Cellular Lipid Metabolism
- Extracellular signal molecules, such as AMPK and ACC, activate the process.
- Lipid Fatty Acids (LCFAs) are transported into the mitochondria.
- CPT1 and CPT2 facilitate the transfer of acylcarnitine into the mitochondrial matrix for beta-oxidation.
- Beta-oxidation breaks down fatty acids, creating Acetyl-CoA, which then enters the citric acid cycle (TCA).
Starvation/Sepsis
- Starvation and sepsis trigger similar responses for lipid utilization.
- Both increase lipolysis in adipose tissue, leading to increased free fatty acids in the blood.
- PPAR-α is a key transcription factor induced by FFAs, promoting further fatty acid oxidation and ketone body production.
- Sepsis can result in decreased PPAR-α levels and impaired fatty acid breakdown.
Peroxisomes
- Peroxisomes contain various enzymes, including catalase, to break down toxic substances, primarily hydrogen peroxide.
- They also handle the breakdown of very long-chain fatty acids via beta oxidation, converting them to medium-chain fatty acids for further mitochondrial processing.
Peroxisomes (Microbodies)
- Peroxisomes were first described by Rhodin (1954) and identified as organelles by De Duve (1967).
- Peroxisomes contain enzymes for hydrogen peroxide metabolism, such as catalase
- Import targeting signals, usually composed of specific amino acid sequences, are important for transporting proteins to peroxisomes.
Mitochondria vs. Peroxisomes
- Mitochondria are double-membrane-bound organelles that contain mtDNA and play a crucial role in oxidative phosphorylation.
- Peroxisomes are single-membrane-bound organelles lacking DNA and are essential for the breakdown of very long-chain fatty acids.
Zellweger Syndrome
- Zellweger syndrome is an inherited disorder caused by mutations in PEX genes.
- This results in inefficient peroxisome function, leading to the accumulation of very long-chain fatty acids (VLCFA) and other abnormal lipids
- Symptoms include characteristic facial deformities, hypotonia, developmental delays, and impaired brain and lung function.
Fatty Acid Oxidation (Beta Oxidation)
- Fatty acid oxidation breaks down fatty acids into acetyl-CoA, generating ATP.
- The process occurs in both mitochondria and peroxisomes
- Different enzymes catalyze each step in the beta-oxidation process.
- Four steps are involved in generating FADH2, NADH, and Acetyl-CoA.
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