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2-Methylbutyryl-L-carnitine is a naturally occurring acylcarnitine that is produced via L-isoleucine metabolism.1 2-Methylbutyryl-L-carnitine levels decrease in OVCAR-8 and adriamycin-resistant OVCAR-8 ovarian cancer cells undergoing ferroptosis induced by erastin (Item No. 17754).2 Plasma levels of 2-methylbutyryl-L-carnitine are elevated in patients with non-alcoholic steatohepatitis (NASH).3 Elevated levels of 2-methylbutyryl-L-carnitine are associated with 2-methylbutyryl-CoA dehydrogenase deficiency (2-MBCDD), also known as short/branched chain acyl-CoA dehydrogenase (SBCAD) deficiency.4
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1. 2-
2. Metabolic consequences of erastin-
3. Plasma metabolomic profile in nonalcoholic fatty liver disease. Metabolism 60(3), 404-413 (2011).
4. Prevalence and mutation analysis of short/branched chain acyl-