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Understanding the Changes in Mitochondrial Morphology through Dynamic and Three-dimensional Fluorescence Micrographs

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ABCD1 deletion-induced mitochondrial dysfunction is corrected by SAHA: implication for adrenoleukodystrophy.

Mauhamad Baarine¹, Craig Beeson, Avtar Singh

¹Department of Pediatrics, Darby Children's Research Institute, Medical University of South Carolina, Charleston, South Carolina, USA.

Journal of Neurochemistry

|November 14, 2014

Summary

Loss of the ABCD1 gene in X-linked Adrenoleukodystrophy disrupts mitochondria, impacting cellular energy and function. Treatment with suberoylanilide hydroxamic acid (SAHA) shows potential to correct these mitochondrial deficits.

Keywords:

X-linked Adrenoleukodystrophy drug therapy gene expression mitochondria peroxisomes suberoylanilide hydroxamic acid

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Area of Science:

Neurobiology
Cellular Biology
Metabolic Disorders

Background:

X-linked Adrenoleukodystrophy (X-ALD) is a neurodegenerative disorder caused by ABCD1 gene mutations, leading to very long chain fatty acid accumulation.
This accumulation disrupts cellular metabolic functions, particularly affecting energy production and homeostasis.

Purpose of the Study:

To investigate the impact of ABCD1 gene deletion on mitochondrial structure and function in oligodendrocytes and astrocytes.
To explore the potential of suberoylanilide hydroxamic acid (SAHA) in correcting these ABCD1-deficiency-induced mitochondrial dysfunctions.

Main Methods:

Silencing of the ABCD1 gene in B12 oligodendrocytes and U87 astrocytes.
Assessment of mitochondrial enzyme activities (electron transport chain, TCA cycle), redox status, membrane potential, ATP levels, and citrate synthase activity.
Treatment of cells with suberoylanilide hydroxamic acid (SAHA).

Main Results:

ABCD1 silencing led to reduced electron transport chain and TCA cycle enzyme activities, dysregulated mitochondrial redox status, and disrupted membrane potential.
Oligodendrocytes showed a greater reduction in ATP levels and citrate synthase activity compared to astrocytes.
SAHA treatment partially corrected the mitochondrial perturbations induced by ABCD1 silencing.

Conclusions:

Peroxisomal dysfunction due to ABCD1 loss significantly impacts mitochondrial integrity and function, highlighting a novel peroxisome-mitochondria interplay.
These findings suggest a potential therapeutic role for SAHA in managing X-ALD by restoring mitochondrial health.