Article
Human disorders of peroxisome metabolism and biogenesis.
Biochimica et biophysica acta - 1 May 2016
Waterham Hans R, Ferdinandusse Sacha, Wanders Ronald J A
Abstract excerpt
Peroxisomes are dynamic organelles that play an essential role in a variety of cellular catabolic and anabolic metabolic pathways, including fatty acid alpha- and beta-oxidation, and plasmalogen and bile acid synthesis. Defects in genes encoding peroxisomal proteins can result in a large variety of peroxisomal disorders either affecting specific metabolic pathways, i.e., the single peroxisomal enzyme...
Topics
- ATPases Associated with Diverse Cellular Activities
- Fatty Acids
- Gene Expression Regulation
- Humans
- Membrane Proteins
- Metabolic Networks and Pathways
- Mutation
- Organelle Biogenesis
- Oxidation-Reduction
- Peroxisomal Disorders
- Peroxisomes
- Plasmalogens
- Protein Isoforms
- Protein Sorting Signals
- Protein Transport
- Signal Transduction
