Results 21 to 30 of about 10,947 (199)

Quantitative analysis of plasma acylcarnitines using gas chromatography chemical ionization mass fragmentography.

Journal of Lipid Research, 1997
A stable isotope dilution gas chromatography chemical ionization mass spectrometry (GC-CI-MS) method was developed for the quantitative profiling of plasma acylcarnitines.
C G Costa, E A Struys, A Bootsma, H J ten Brink, L Dorland, I Tavares de Almeida, M Duran, C Jakobs   +7 more
doaj   +1 more source

Diagnostic Challenges in Late Onset Multiple Acyl-CoA Dehydrogenase Deficiency: Clinical, Morphological, and Genetic Aspects

Frontiers in Neurology, 2022
BackgroundMultiple acyl-CoA dehydrogenase deficiency (MADD) is an autosomal recessive disorder of fatty acid oxidation due to deficiency of the mitochondrial electron transfer chain.
Antonino Lupica, Rosaria Oteri, Sara Volta, Daniele Ghezzi, Daniele Ghezzi, Selene Francesca Anna Drago, Carmelo Rodolico, Olimpia Musumeci, Antonio Toscano   +8 more
doaj   +1 more source

Relation of Whole Blood Amino Acid and Acylcarnitine Metabolome to Age, Sex, BMI, Puberty, and Metabolic Markers in Children and Adolescents

Metabolites, 2020
Background: Changes in the metabolic fingerprint of blood during child growth and development are a largely under-investigated area of research. The examination of such aspects requires a cohort of healthy children and adolescents who have been subjected
Josephin Hirschel, Mandy Vogel, Ronny Baber, Antje Garten, Carl Beuchel, Yvonne Dietz, Julia Dittrich, Antje Körner, Wieland Kiess, Uta Ceglarek   +9 more
doaj   +1 more source

Acylcarnitines: Role in brain

Progress in Lipid Research, 2010
l-carnitine is present in mammalian cells as free carnitine and acylcarnitines. The acylcarnitine profile has been shown to be useful in identifying inborn errors of metabolism and to be altered under different metabolic conditions. While carnitine's most widely known function is its involvement in beta-oxidation of fatty acids, it may also have other ...
Department of Food Science and Human Nutrition, University of Florida, Gainesville, FL, United States ( host institution ), Jones, Lauren L. ( author ), McDonald, David A. ( author ), Borum, Peggy R. ( author )   +3 more
openaire   +3 more sources

Function, Detection and Alteration of Acylcarnitine Metabolism in Hepatocellular Carcinoma

Metabolites, 2019
Acylcarnitines play an essential role in regulating the balance of intracellular sugar and lipid metabolism. They serve as carriers to transport activated long-chain fatty acids into mitochondria for β-oxidation as a major source of energy for cell ...
Shangfu Li, Dan Gao, Yuyang Jiang
doaj   +1 more source

Acylcarnitine profiling by low-resolution LC-MS.

PLoS ONE, 2019
Acylcarnitines are fatty acyl esters of L-carnitine and facilitate the entry of long-chain fatty acids into mitochondria via the carnitine shuttle, where they are metabolized via ß-oxidation.
David Meierhofer
doaj   +1 more source

Serum Metabolite Profile Associated with Sex-Dependent Visceral Adiposity Index and Low Bone Mineral Density in a Mexican Population

Metabolites, 2021
Recent evidence shows that obesity correlates negatively with bone mass. However, traditional anthropometric measures such as body mass index could not discriminate visceral adipose tissue from subcutaneous adipose tissue.
Berenice Palacios-González, Guadalupe León-Reyes, Berenice Rivera-Paredez, Isabel Ibarra-González, Marcela Vela-Amieva, Yvonne N. Flores, Samuel Canizales-Quinteros, Jorge Salmerón, Rafael Velázquez-Cruz   +8 more
doaj   +1 more source

A newborn screening approach to diagnose 3‐hydroxy‐3‐methylglutaryl‐CoA lyase deficiency

JIMD Reports, 2020
3‐Hydroxy‐3‐methylglutaryl‐coenzyme A lyase deficiency (HMGCLD) is a rare autosomal recessively inherited metabolic disorder. Patients suffer from avoidable neurologically devastating metabolic decompensations and thus would benefit from newborn ...
Jan Václavík, Lucie Mádrová, Štěpán Kouřil, Julie de Sousa, Radana Brumarová, Hana Janečková, Jaroslava Jáčová, David Friedecký, Mária Knapková, Leo A. J. Kluijtmans, Sarah C. Grünert, Frédéric M. Vaz, Nils Janzen, Ronald J. A. Wanders, Ron A. Wevers, Tomáš Adam   +15 more
doaj   +1 more source

Simultaneously quantifying hundreds of acylcarnitines in multiple biological matrices within ten minutes using ultrahigh-performance liquid-chromatography and tandem mass spectrometry

Journal of Pharmaceutical Analysis
Acylcarnitines are metabolic intermediates of fatty acids and branched-chain amino acids having vital biofunctions and pathophysiological significances.
Jingxian Zhang, Qinsheng Chen, Lianglong Zhang, Biru Shi, Men Yu, Qingxia Huang, Huiru Tang   +6 more
doaj   +1 more source

NMR Characterization of Long-Chain Fatty Acylcarnitine Binding to the Mitochondrial Carnitine/Acylcarnitine Carrier

International Journal of Molecular Sciences, 2022
The mitochondrial carnitine/acylcarnitine carrier (CAC) transports short-, medium- and long-carbon chain acylcarnitines across the mitochondrial inner membrane in exchange for carnitine. How CAC recognizes the substrates with various fatty acyl groups, especially long-chain fatty acyl groups, remains unclear. Here, using nuclear magnetic resonance (NMR)
Ningning Zhang, Xiaopu Jia, Shuai Fan, Bin Wu, Shuqing Wang, Bo OuYang   +5 more
openaire   +2 more sources