Insights into the Structure and Function of the Pex1/Pex6 AAA-ATPase in Peroxisome Homeostasis
| Autor: | Judy, Ryan M, Sheedy, Connor J, Gardner, Brooke M |
|---|---|
| Rok vydání: | 2022 |
| Předmět: |
Adenosine Triphosphatases
PEX1 1.1 Normal biological development and functioning Membrane Proteins PEX26 peroxisomes translocation PEX6 Rare Diseases Underpinning research Peroxisomes Humans Homeostasis ATPases Associated with Diverse Cellular Activities Generic health relevance organelle biogenesis AAA-ATPase |
| Zdroj: | Cells, vol 11, iss 13 |
| Popis: | The AAA-ATPases Pex1 and Pex6 are required for the formation and maintenance of peroxisomes, membrane-bound organelles that harbor enzymes for specialized metabolism. Together, Pex1 and Pex6 form a heterohexameric AAA-ATPase capable of unfolding substrate proteins via processive threading through a central pore. Here, we review the proposed roles for Pex1/Pex6 in peroxisome biogenesis and degradation, discussing how the unfolding of potential substrates contributes to peroxisome homeostasis. We also consider how advances in cryo-EM, computational structure prediction, and mechanisms of related ATPases are improving our understanding of how Pex1/Pex6 converts ATP hydrolysis into mechanical force. Since mutations in PEX1 and PEX6 cause the majority of known cases of peroxisome biogenesis disorders such as Zellweger syndrome, insights into Pex1/Pex6 structure and function are important for understanding peroxisomes in human health and disease. |
| Databáze: | OpenAIRE |
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