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Analysis of DepMAP data revealed that the knockout effect of peroxisome genes (i.e. PEX1) had no significant correlation with the expression of either HIF1A or MYC while SCD knockout effect was ...
aState Key Laboratory of Medical Molecular Biology, Department of Biochemistry and Molecular Biology, Institute of Basic Medical Sciences Chinese Academy of Medical Sciences, School of Basic Medicine ...
The leading player in this process is the peroxisomal AAA-ATPase complex Pex1/Pex6, often called a “biological nanomachine.” It unwraps and unfolds these spent proteins to be reused or disposed of.
Scientists at Münster University used cryogenic electron microscopy to study the atomic-level processes of peroxisomal AAA-ATPase complex Pex1/Pex6, crucial for detoxification and fat metabolism in ...
The different PEX genes are PEX1, PEX2, PEX5, PEX6, PEX12, PEX26 among others. Around 60% of ZSD patients have biallelic PEX1 mutations, and nearly 90 distinct PEX1 mutations have been identified ...
We find that latent activation of the yeast pexophagy receptor Atg36 by the casein kinase Hrr25 in rich media is repressed by the ATPase activity of Pex1/6, the catalytic subunits of the exportomer ...
In a typical PBD-ZSD family, the parents may each be a carrier of a mutation in a gene, such as PEX1. The misspelt version of the PEX1 gene in both parents does not impact their health and has no ...
Currently, there is no specific, effective treatment. Here, we investigated the effects of allogeneic hematopoietic stem cell transplantation (allo-HSCT) on PEX1-related ZSD. The suspected clinical ...
2528G>A (p.G843D) mutation in the PEX1 gene (PEX1-G843D), which results in impaired import of peroxisomal matrix proteins and, consequently, defective peroxisomal functions. A recent study suggested ...
Peer ReviewDownload a summary of the editorial decision process including editorial decision letters, reviewer comments and author responses to feedback. This study reveals that depletion of TMEM135 ...
The collaborators propose to test the gene therapy targeting retinal photoreceptor cells in PEX1 animal models to study recovery of peroxisomal function,” says Inès Holzbaur, Managing Partner ...
Peroxisomes are conserved organelles of eukaryotic cells with important roles in cellular metabolism, human health, redox homeostasis, as well as intracellular metabolite transfer and signaling. We ...
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