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Oxidative Stress in Patients with X-Linked Adrenoleukodystrophy

Cellular and Molecular Neurobiology volume 36, pages 497–512 (2016)Cite this article

Abstract

X-linked adrenoleukodystrophy (X-ALD) is the most frequent peroxisomal disorder that is characterized by progressive demyelination of the white matter, adrenal insufficiency, and accumulation of very long-chain fatty acids in body fluid and tissues. This disorder is clinically heterogeneous with seven different phenotypes in male patients and five phenotypes in female carriers. An ultimate treatment for X-ALD is not available. Depending on the rate of the disease progression and the degree of an individual handicap, special needs and challenges vary greatly. The exact mechanisms underlying the pathophysiology of this multifactorial neurodegenerative disorder remains obscure. Previous studies has been related oxidative stress with the pathogenesis of several disease that affecting the central nervous system, such as neurodegenerative disease, epilepsy, multiple sclerosis, Alzheimer, and Parkinson diseases. In addition, oxidative damage has been observed in various in vivo and in vitro studies with inborn errors of metabolism, including X-ALD. In this context, this review is focused on oxidative stress in X-ALD, with emphasis on studies using biological samples from patients affected by this disease.

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Fig. 1

Abbreviations

AASA:

Aminoadipic semialdehyde

ABCD1:

ATP-binding cassette (ABC) transporter subfamily D member 1

ABAP:

2,2′-Azo-bis-(2-aminidinopropane)

AMN:

Adrenomyeloneuropathy

ATP:

Adenosine triphosfate

BMT:

Bone marrow transplant

C22:0:

Docosanoic acid

C24:0:

Tetracosanoic acid

C26:0:

Hexacosanoic acid

Ca2+ :

Calcium

CAT:

Catalase

CCER:

Cerebral childhood ALD

CEL:

Carboxyethyl-lysine

CML:

Carboxylmethyl-lysine

CNS:

Central nervous system

DNA:

Deoxyribonucleic acid

GPx:

Glutathione peroxidase

GSA:

Glutamic semialdehyde

GSH:

Glutathione

GSHRd:

Glutathione reductase

GSSG:

Oxidized glutathione

H2DCFDA:

6-Carboxy 2′,7′-dichlorodihydrofluorescein

H2O2 :

Hydrogen peroxide

HNE:

4-Hydroxynonenal

HO-1:

Hemoxygenase-1

HPLC:

High-performance liquid chromatography

HSCT:

Hematopoietic stem cell transplantation

HTZ:

Heterozygote or female carriers

IEM:

Inborn errors of metabolism

IFN-γ:

Interferon gamma

IL-12:

Interleukin 12

IL-1β:

Interleukin 1 beta

iNOS:

Inducible nitric oxide synthase

LA:

Lipoic acid

LDL:

Low-density lipoprotein

LO:

Lorenzo’s oil

MDA:

Malondialdehyde

MDAL:

Malondialdehyde–lysine

MnSOD:

Manganese–superoxide dismutase

MRI:

Magnetic resonance image

mtDNA:

Mitochondrial DNA

NAC:

N-acetyl-cystein

NaPA:

Sodium phenylacetate

NO:

Oxide nitric

O2 :

Oxygen

O •−2 :

Superoxide radical

OH• :

Hydroxyl radical

ONOO− :

Peroxynitrite

OXPHOS:

Oxidative phosphorylation

PlsEtn:

Plasmenylethanolamine

PUFA:

Polyunsaturated fatty acid

RNS:

Reactive nitrogen species

ROS:

Reactive oxygen species

RS:

Reactive species

SOD:

Superoxide dismutase

TAR:

Total antioxidant reactivity

TAS:

Total antioxidant status

TBA-RS:

Thiobarbituric acid reactive species

TNF-α:

Tumor necrosis factor alpha

TRAP:

Total radical-trapping antioxidant potential

VLCFA:

Very long chain fatty acidy

VPA:

Valproic acid

X-ALD:

X-linked adrenoleukodystrophy

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Acknowledgments

The authors were supported by grants from the Brazilian Foundation Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES) (Grant No. 007481/2011-13), Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) and Fundo de Incentivo à Pesquisa e Eventos (FIPE/HCPA) (Project Nos. 01-0432, 10-0482 and 13-0247).

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Authors and Affiliations

  1. Programa de Pós-Graduação em Ciências Farmacêuticas, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil

    Marion Deon & Carmen Vargas

  2. Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil

    Desirèe P. Marchetti, Bruna Donida, Moacir Wajner & Carmen Vargas

  3. Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre (HCPA), Rua Ramiro Barcelos, 2350, Porto Alegre, RS, CEP 90035-003, Brazil

    Marion Deon, Desirèe P. Marchetti, Bruna Donida, Moacir Wajner & Carmen Vargas

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  1. Marion Deon
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Deon, M., Marchetti, D.P., Donida, B. et al. Oxidative Stress in Patients with X-Linked Adrenoleukodystrophy. Cell Mol Neurobiol 36, 497–512 (2016). https://doi.org/10.1007/s10571-015-0234-2

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Keywords

  • X-linked adrenoleukodystrophy
  • Oxidative stress
  • Antioxidants
  • Oxidative damage biomarkers