Treatment effect of coenzyme Q10 and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease

Secondary coenzyme Q10 deficiency and oxidative stress in cultured fibroblasts from patients with riboflavin responsive multiple Acyl-CoA dehydrogenation deficiency

(2013) N. Cornelius et al.   HUMAN MOLECULAR GENETICS

Characterization of CoQ10 biosynthesis in fibroblasts of patients with primary and secondary CoQ10 deficiency

(2013) Nuria Buján et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Inflammation-induced dysfunction of the low-density lipoprotein receptor-related protein-1 at the blood–brain barrier: Protection by the antioxidant N-acetylcysteine

(2012) Michelle A. Erickson et al.   BRAIN BEHAVIOR AND IMMUNITY

δ-Tocopherol Reduces Lipid Accumulation in Niemann-Pick Type C1 and Wolman Cholesterol Storage Disorders

(2012) Miao Xu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Lysosomal storage disorders: The cellular impact of lysosomal dysfunction

(2012) Frances M. Platt et al.   JOURNAL OF CELL BIOLOGY

Analysis of coenzyme Q10 in lymphocytes by HPLC–MS/MS

(2012) A. Arias et al.   JOURNAL OF CHROMATOGRAPHY B-ANALYTICAL TECHNOLOGIES IN THE BIOMEDICAL AND LIFE SCIENCES

Antioxidants halt axonal degeneration in a mouse model of X-adrenoleukodystrophy

(2011) Jone López-Erauskin et al.   ANNALS OF NEUROLOGY

Mucopolysaccharidosis Type III (Sanfilippo Syndrome): Emerging Treatment Strategies

(2011) J. de Ruijter et al.   CURRENT PHARMACEUTICAL BIOTECHNOLOGY

Genistein supplementation in patients affected by Sanfilippo disease

(2011) Verónica Delgadillo et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Oxidative stress in Niemann–Pick disease, type C

(2010) Rao Fu et al.   MOLECULAR GENETICS AND METABOLISM

A galactose-free diet enriched in soy isoflavones and antioxidants results in delayed onset of symptoms of Krabbe disease in twitcher mice

(2010) Giovanna Pannuzzo et al.   MOLECULAR GENETICS AND METABOLISM

Safe, Efficient, and Reproducible Gene Therapy of the Brain in the Dog Models of Sanfilippo and Hurler Syndromes

(2010) N Matthew Ellinwood et al.   MOLECULAR THERAPY

Coenzyme Q deficiency triggers mitochondria degradation by mitophagy

(2009) Ángeles Rodríguez-Hernández et al.   Autophagy

Sanfilippo syndrome: A mini-review

(2008) M. J. Valstar et al.   JOURNAL OF INHERITED METABOLIC DISEASE