Defective cholesterol metabolism in amyotrophic lateral sclerosis

• Published in: Journal of lipid research Vol. 58; no. 1; pp. 267 - 278
• Main Authors: Abdel-Khalik, Jonas, Yutuc, Eylan, Crick, Peter J., Gustafsson, Jan-Åke, Warner, Margaret, Roman, Gustavo, Talbot, Kevin, Gray, Elizabeth, Griffiths, William J., Turner, Martin R., Wang, Yuqin
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.01.2017; Journal of Lipid Research; The American Society for Biochemistry and Molecular Biology; Elsevier
• Subjects: Acids; Aged; Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - blood; Amyotrophic Lateral Sclerosis - cerebrospinal fluid; Amyotrophic Lateral Sclerosis - pathology; Bile; Bile Acids and Salts - blood; Bile Acids and Salts - cerebrospinal fluid; Bile Acids and Salts - isolation & purification; bile acids and salts/biosynthesis; brain lipids; Central nervous system; Central Nervous System - metabolism; Central Nervous System - pathology; Cerebrospinal fluid; cholestenoic acids; Cholesterol; Cholesterol - blood; Cholesterol - cerebrospinal fluid; Cholesterol - isolation & purification; cytochrome P450; Female; Humans; Lipid metabolism; Lipids - blood; Lipids - cerebrospinal fluid; Lipids - isolation & purification; Male; mass spectrometry; Metabolites; Middle Aged; Nerve Degeneration - blood; Nerve Degeneration - cerebrospinal fluid; Nerve Degeneration - pathology; Neurodegeneration; neurodeneneration; Neurons - metabolism; Neurons - pathology; Neuroprotection; nuclear receptors/LXR; oxysterols; Patient-Oriented and Epidemiological Research; Sterols; cholestenoic acids; brain lipids; neurodeneneration; bile acids and salts/biosynthesis; oxysterols; nuclear receptors/LXR; mass spectrometry; cytochrome P450

As neurons die, cholesterol is released in the central nervous system (CNS); hence, this sterol and its metabolites may represent a biomarker of neurodegeneration, including in amyotrophic lateral sclerosis (ALS), in which altered cholesterol levels have been linked to prognosis. More than 40 differ...