Lipoprotein[a] is not present in the plasma of patients with some peroxisomal disorders

Hoek, Ytje Y. van der; Wanders, R. J. A.; Ende, A. E. Van Den; Kraft, H. G.; Gabel, Brent R.; Kastelein, J.J.P.; Koschinsky, Marlys L.

doi:10.1016/s0022-2275(20)37179-0

Cited by 5 publications

(1 citation statement)

References 42 publications

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“…Increased lipolysis through enhanced lipoprotein or hormone-sensitive lipase activity was also less likely, given that this was a liver-specific KO model. Limited data suggest that very-low-density lipoprotein secretion is not altered in patients with peroxisome biogenesis disorders [ 42 ]. Although expression of genes encoding for enzymes controlling β-oxidation was increased in Pex16 KO mice, we cannot exclude reduced β-oxidation in these mice.…”

Section: Discussionmentioning

confidence: 99%

Loss of functional peroxisomes leads to increased mitochondrial biogenesis and reduced autophagy that preserve mitochondrial function

Chi

Lee

Leung

et al. 2023

Cell. Mol. Life Sci.

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Peroxisomes are essential for mitochondrial health, as the absence of peroxisomes leads to altered mitochondria. However, it is unclear whether the changes in mitochondria are a function of preserving cellular function or a response to cellular damage caused by the absence of peroxisomes. To address this, we developed conditional hepatocyte-specific Pex16 deficient (Pex16 KO) mice that develop peroxisome loss and subjected them to a low-protein diet to induce metabolic stress. Loss of PEX16 in hepatocytes led to increased biogenesis of small mitochondria and reduced autophagy flux but with preserved capacity for respiration and ATP capacity. Metabolic stress induced by low protein feeding led to mitochondrial dysfunction in Pex16 KO mice and impaired biogenesis. Activation of PPARα partially corrected these mitochondrial disturbances, despite the absence of peroxisomes. The findings of this study demonstrate that the absence of peroxisomes in hepatocytes results in a concerted effort to preserve mitochondrial function, including increased mitochondrial biogenesis, altered morphology, and modified autophagy activity. Our study underscores the relationship between peroxisomes and mitochondria in regulating the hepatic metabolic responses to nutritional stressors.

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Section: Discussionmentioning

confidence: 99%

Loss of functional peroxisomes leads to increased mitochondrial biogenesis and reduced autophagy that preserve mitochondrial function

Chi

Lee

Leung

et al. 2023

Cell. Mol. Life Sci.

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Functions of plasmalogen lipids in health and disease

Braverman

Moser

2012

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Diseas

864

890

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Plasmalogens are a unique class of membrane glycerophospholipids containing a fatty alcohol with a vinyl-ether bond at the sn-1 position, and enriched in polyunsaturated fatty acids at the sn-2 position of the glycerol backbone. These two features provide novel properties to these compounds. Although plasmalogens represent up to 20% of the total phospholipid mass in humans their physiological roles have been challenging to identify, and are likely to be particular to different tissues, metabolic processes and developmental stages. Their biosynthesis starts in peroxisomes, and defects at these steps cause the malformation syndrome, Rhizomelic Chondrodysplasia Punctata (RCDP). The RCDP phenotype predicts developmental roles for plasmalogens in bone, brain, lens, lung, kidney and heart. Recent studies have revealed secondary plasmalogen deficiencies associated with more common disorders and allow us to tease out additional pathways dependent on plasmalogen functions. In this review, we present current knowledge of plasmalogen biology in health and disease.

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A metabolomic map of Zellweger spectrum disorders reveals novel disease biomarkers

Wangler

Hubert

Donti

et al. 2018

Genetics in Medicine

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Lipoprotein[a] is not present in the plasma of patients with some peroxisomal disorders

Cited by 5 publications

References 42 publications

Loss of functional peroxisomes leads to increased mitochondrial biogenesis and reduced autophagy that preserve mitochondrial function

Loss of functional peroxisomes leads to increased mitochondrial biogenesis and reduced autophagy that preserve mitochondrial function

Functions of plasmalogen lipids in health and disease

A metabolomic map of Zellweger spectrum disorders reveals novel disease biomarkers

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