title: Oxidative Stress in Patients with X-Linked Adrenoleukodystrophy.
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.
authors.affiliation: Programa de Pós-Graduação em Ciências Farmacêuticas, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil. marion_deon@yahoo.com.br.|Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre (HCPA), Rua Ramiro Barcelos, 2350, Porto Alegre, RS, CEP 90035-003, Brazil. marion_deon@yahoo.com.br.
authors.forename: Marion
authors.initials: M
authors.lastname: Deon
authors.affiliation: Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.|Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre (HCPA), Rua Ramiro Barcelos, 2350, Porto Alegre, RS, CEP 90035-003, Brazil.
authors.forename: Desirèe P
authors.initials: DP
authors.lastname: Marchetti
authors.affiliation: Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.|Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre (HCPA), Rua Ramiro Barcelos, 2350, Porto Alegre, RS, CEP 90035-003, Brazil.
authors.forename: Bruna
authors.initials: B
authors.lastname: Donida
authors.affiliation: Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.|Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre (HCPA), Rua Ramiro Barcelos, 2350, Porto Alegre, RS, CEP 90035-003, Brazil.
authors.forename: Moacir
authors.initials: M
authors.lastname: Wajner
authors.affiliation: Programa de Pós-Graduação em Ciências Farmacêuticas, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil. crvargas@hcpa.edu.br.|Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil. crvargas@hcpa.edu.br.|Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre (HCPA), Rua Ramiro Barcelos, 2350, Porto Alegre, RS, CEP 90035-003, Brazil. crvargas@hcpa.edu.br.
authors.forename: Carmen
authors.initials: C
authors.lastname: Vargas
mesh_terms: D000326:Adrenoleukodystrophy / Q000473:pathology* / Q000628:therapy; D000975:Antioxidants / Q000378:metabolism; D015415:Biomarkers / Q000378:metabolism; D005609:Free Radicals / Q000378:metabolism; D006801:Humans; D008954:Models, Biological; D018384:Oxidative Stress
chemical_list: D000975:Antioxidants; D015415:Biomarkers; D005609:Free Radicals
keywords: Antioxidants; Oxidative damage biomarkers; Oxidative stress; X-linked adrenoleukodystrophy