Hydroxylated long-chain acylcarnitines are biomarkers of mitochondrial myopathy
نویسندگان
چکیده
منابع مشابه
Some micellar properties of long-chain acylcarnitines.
The acid dissociation constants of long-chain esters of carnitine (5-hydroxy-~,trimethylammonium-butyrate) above the critical micelle concentration were determined potentiometrieally at several concentrations of added KC1. As the degree of protonation 5 increases the apparent pK values decrease owing to the increased positive charge on the micelle. The difference in pK between the neutral (zwit...
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BACKGROUND AND PURPOSE In some pathological conditions carnitine concentration is high while in others it is low. In both cases,cardiac arrhythmias can occur and lead to sudden cardiac death. It has been proposed that in ischaemia, acylcarnitine (acyl-CAR), but not carnitine, is involved in arrhythmias through modulation of ionic currents. We studied the effects of acyl-CARs on hERG, K(IR)2.1 a...
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The role of mitochondrial energy metabolism in maintaining lung function is not understood. We previously observed reduced lung function in mice lacking the fatty acid oxidation enzyme long-chain acyl-CoA dehydrogenase (LCAD). Here, we demonstrate that long-chain acylcarnitines, a class of lipids secreted by mitochondria when metabolism is inhibited, accumulate at the air-fluid interface in LCA...
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Equine atypical myopathy (AM) is caused by hypoglycin A intoxication and is characterized by a high fatality rate. Predictive estimation of survival in AM horses is necessary to prevent unnecessary suffering of animals that are unlikely to survive and to focus supportive therapy on horses with a possible favourable prognosis of survival. We hypothesized that outcome may be predicted early in th...
متن کاملMitochondrial processes are impaired in hereditary inclusion body myopathy.
Hereditary inclusion body myopathy (HIBM) is an adult onset, slowly progressive distal and proximal myopathy. Although the causing gene, GNE, encodes for a key enzyme in the biosynthesis of sialic acid, its primary function in HIBM remains unknown. To elucidate the pathological mechanisms leading from the mutated GNE to the HIBM phenotype, we attempted to identify and characterize early occurri...
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ژورنال
عنوان ژورنال: The Journal of Clinical Endocrinology & Metabolism
سال: 2019
ISSN: 0021-972X,1945-7197
DOI: 10.1210/jc.2019-00721