Zebrafish model of human Zellweger syndrome reveals organ specific accumulation of distinct fatty acid species and widespread gene expression changes

Abstract excerpt

<h4>ABSTRACT</h4> In Zellweger syndrome (ZS), lack of peroxisome function causes physiological and developmental abnormalities in many organs such as the brain, liver, muscles, and kidneys, but little is known about the exact pathogenic mechanism. By disrupting the zebrafish pex2 gene, we established a disease model for ZS and found that it exhibits a pathological condition and metabolic failures similar to that...

Topics

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• ATPases Associated with Diverse Cellular Activities
• Bile Acids and Salts
• Disease Management
• Metabolism and Genetic Disorders
• Mutation
• Peroxisome Proliferator-Activated Receptors
• Peroxisomes
• Zellweger Syndrome

Identifiers and source

Literature Corpus work

ffd9db98-c3f2-521e-b7d7-0b97e2fc1b7c

DOI

10.1101/2021.01.03.425169