Peroxisomes and receptors in health and disease

Question 1

What is the core conserved metabolic pathway of peroxisomes?

Answer 1

B-oxidation - long and branched fatty acid shortening

Question 2

What is the metabolic pathway of peroxisomes used in?

Answer 2

Degradative and biosynthetic pathways

Question 3

What are peroxisome biogenesis disorders?

Answer 3

Severe multisystem disorders caused by the failure to assemble peroxisomes properly and the loss of metabolic pathways.

Question 4

name a peroxisome biogenesis disorders

Answer 4

Zellweger Syndrome

Question 5

What is X-linked adrenoleukodystrophy (X-ALD)?

Answer 5

A single gene defect that affects B-oxidation causing an accumulation of long chain fatty acids and resulting in the demyelination of neurones.

Question 6

What is primary hyperoxaluria type 1 (PH1)?

Answer 6

Deficiency of peroxisomal alanine glycolate amino transferase resulting in high levels of calcium oxalate leading to renal failure. Most have the AGT enzyme missing but in some it is mislocalised to the mitochondria.

Question 7

What is required for infection in some plant pathogenic fungi?

Answer 7

The formation of specialised structures called appressoria which have thick melanised walls.

Question 8

What can go wrong to cause the different spectrums of ZS?

Answer 8

The receptor, components of the docking or recycling complexes are defective.
Peroxisomes are absent or empty.
Enzymes are mislocalised if the peroxisome is not present.