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Peroxisomes of the Brain: Distribution, Functions, and Associated Diseases

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Abstract

Peroxisomes are versatile cell organelles that exhibit a repertoire of organism and cell-type dependent functions. The presence of oxidases and antioxidant enzymes is a characteristic feature of these organelles. The role of peroxisomes in various cell types in human health and disease is under investigation. Defects in the biogenesis of the organelle and its function lead to severe debilitating disorders. In this manuscript, we discuss the distribution and functions of peroxisomes in the nervous system and especially in the brain cells. The important peroxisomal functions in these cells and their role in the pathology of associated disorders such as neurodegeneration are highlighted in recent studies. Although the cause of the pathogenesis of these disorders is still not clearly understood, emerging evidence supports a crucial role of peroxisomes. In this review, we discuss research highlighting the role of peroxisomes in brain development and its function. We also provide an overview of the major findings in recent years that highlight the role of peroxisome dysfunction in various associated diseases.

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Abbreviations

ACOX:

Acyl CoA oxidase

AD:

Alzheimer’s disease

ALS:

Amyotrophic lateral sclerosis

CNS:

Central nervous system

D-AAO:

D-amino acid oxidase

DBPD:

Bifunctional protein

DHA:

Docosahexanoic acid

DHAPAT:

Dihydroxyacetone phosphate acyl-transferase

DHCA:

Dihydroxycholestanoic acid

DS:

Down syndrome

EAE:

Experimental autoimmune encephalomyelitis

LBP:

L-bifunctional protein

MFP:

Multifunctional protein

MS:

Multiple sclerosis

MYOC:

Myocilin

NPC:

Niemann-Pick type C

PBDs:

Peroxisome biogenesis disorders

PD:

Parkinson’s disease

PEDs:

Peroxisome enzyme deficiencies

PHYH:

Phytanoyl-CoA hydroxylase

PNS:

Peripheral nervous system

PTS:

Peroxisome targeting signal

PUFA:

Polyunsaturated fatty acid

RCDP:

Rhizomelic chondrodysplasia punctata

ROS:

Reactive oxygen species

THCA:

Trihydroxycholestanoic acid

TM:

Trabecular meshwork

VLCFAs:

Very long chain fatty acids

X-ALD:

X-linked adrenoleukodystrophy

ZS:

Zellweger syndrome

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Acknowledgments

Our sincere apologies to all our colleagues for any publication not being cited due to space limitation.

Funding

This work was supported by Department of Biotechnology (DBT), Government of India [BT/PR25097/NER/95/1013/2017] Grant.

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Correspondence to Shirisha Nagotu.

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The authors declare that they have no conflict of interest.

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Deb, R., Joshi, N. & Nagotu, S. Peroxisomes of the Brain: Distribution, Functions, and Associated Diseases. Neurotox Res 39, 986–1006 (2021). https://doi.org/10.1007/s12640-020-00323-9

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  • DOI: https://doi.org/10.1007/s12640-020-00323-9

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