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acetylcarnitine and Amino Acid Metabolism Disorders, Inborn

acetylcarnitine has been researched along with Amino Acid Metabolism Disorders, Inborn in 17 studies

Acetylcarnitine: An acetic acid ester of CARNITINE that facilitates movement of ACETYL COA into the matrices of mammalian MITOCHONDRIA during the oxidation of FATTY ACIDS.

Research Excerpts

ExcerptRelevanceReference
"Acetylcarnitine was detected in the urine only when sufficient carnitine was supplemented."1.29Effect of carnitine administration on glycine metabolism in patients with isovaleric acidemia: significance of acetylcarnitine determination to estimate the proper carnitine dose. ( Ito, T; Itoh, T; Kidouchi, K; Mizuguchi, K; Ohba, S; Sugiyama, N; Yamaguchi, S, 1996)

Research

Studies (17)

TimeframeStudies, this research(%)All Research%
pre-19903 (17.65)18.7374
1990's7 (41.18)18.2507
2000's1 (5.88)29.6817
2010's4 (23.53)24.3611
2020's2 (11.76)2.80

Authors

AuthorsStudies
Wang, F1
Liang, L1
Ling, S1
Yu, Y1
Chen, T1
Xu, F1
Gong, Z1
Han, L1
Mütze, U1
Henze, L1
Schröter, J1
Gleich, F2
Lindner, M1
Grünert, SC1
Spiekerkoetter, U1
Santer, R1
Thimm, E1
Ensenauer, R1
Weigel, J1
Beblo, S1
Arélin, M1
Hennermann, JB1
Marquardt, I1
Freisinger, P1
Krämer, J1
Dieckmann, A1
Weinhold, N1
Schiergens, KA1
Maier, EM1
Hoffmann, GF1
Garbade, SF1
Kölker, S2
Keller, R1
Chrastina, P1
Pavlíková, M1
Gouveia, S1
Ribes, A1
Blom, HJ1
Baumgartner, MR1
Bártl, J1
Dionisi-Vici, C1
Morris, AA1
Kožich, V1
Huemer, M1
Barić, I1
Ben-Omran, T1
Blasco-Alonso, J1
Bueno Delgado, MA1
Carducci, C1
Cassanello, M1
Cerone, R1
Couce, ML1
Crushell, E1
Delgado Pecellin, C1
Dulin, E1
Espada, M1
Ferino, G1
Fingerhut, R1
Garcia Jimenez, I1
Gonzalez Gallego, I1
González-Irazabal, Y1
Gramer, G1
Juan Fita, MJ1
Karg, E1
Klein, J1
Konstantopoulou, V1
la Marca, G1
Leão Teles, E1
Leuzzi, V1
Lilliu, F1
Lopez, RM1
Lund, AM1
Mayne, P1
Meavilla, S1
Moat, SJ1
Okun, JG1
Pasquini, E1
Pedron-Giner, CC1
Racz, GZ1
Ruiz Gomez, MA1
Vilarinho, L1
Yahyaoui, R1
Zerjav Tansek, M1
Zetterström, RH1
Zeyda, M1
Wu, SN1
Han, LS1
Ye, J1
Qiu, WJ1
Zhang, HW1
Gao, XL1
Wang, Y1
Li, XY1
Xu, H1
Gu, XF1
Al-Dirbashi, OY1
McIntosh, N1
Chakraborty, P1
Hori, T1
Fukao, T1
Kobayashi, H1
Teramoto, T1
Takayanagi, M1
Hasegawa, Y1
Yasuno, T1
Yamaguchi, S3
Kondo, N1
Wikoff, WR1
Gangoiti, JA1
Barshop, BA1
Siuzdak, G1
Roe, CR2
Hoppel, CL1
Stacey, TE1
Chalmers, RA1
Tracey, BM1
Millington, DS2
Van Hove, JL1
Kahler, SG1
Roe, DS1
Chace, DH1
Heales, SJ1
Poorthuis, BJ1
Jille-Vlcková, T1
Onkenhout, W1
Shigematsu, Y1
Hata, I1
Nakai, A1
Kikawa, Y1
Sudo, M1
Tanaka, Y1
Jakobs, C2
Itoh, T1
Ito, T1
Ohba, S1
Sugiyama, N2
Mizuguchi, K1
Kidouchi, K2
Mawal, YR1
Rama Rao, KV1
Qureshi, IA2
Rao, KV1
Sakuma, T1
Penn, D1
Schmidt-Sommerfeld, E1
Bieber, LL1
Morishita, H1
Kobayashi, M1
Wada, Y1
Nohara, D1

Other Studies

17 other studies available for acetylcarnitine and Amino Acid Metabolism Disorders, Inborn

ArticleYear
Clinical characteristics and genotype analysis of five infants with cblX type of methylmalonic acidemia.
    Zhejiang da xue xue bao. Yi xue ban = Journal of Zhejiang University. Medical sciences, 2022, Jun-25, Volume: 51, Issue:3

    Topics: Acetylcarnitine; Amino Acid Metabolism, Inborn Errors; China; Drug Resistant Epilepsy; Female; Genot

2022
Isovaleric aciduria identified by newborn screening: Strategies to predict disease severity and stratify treatment.
    Journal of inherited metabolic disease, 2023, Volume: 46, Issue:6

    Topics: Acetylcarnitine; Amino Acid Metabolism, Inborn Errors; Child; Genotype; Glycine; Humans; Infant, New

2023
Newborn screening for homocystinurias: Recent recommendations versus current practice.
    Journal of inherited metabolic disease, 2019, Volume: 42, Issue:1

    Topics: Acetylcarnitine; Amino Acid Metabolism, Inborn Errors; Carnitine; Female; Glycine N-Methyltransferas

2019
[Analysis of propionylcarnitine in blood and methylmalonic acid in urine of 162 patients with methylmalonic acidemia].
    Zhonghua yi xue za zhi, 2013, Feb-26, Volume: 93, Issue:8

    Topics: Acetylcarnitine; Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Carnitine; Case-Control St

2013
Quantification of 2-methylcitric acid in dried blood spots improves newborn screening for propionic and methylmalonic acidemias.
    Journal of medical screening, 2017, Volume: 24, Issue:2

    Topics: Acetylcarnitine; Algorithms; Amino Acid Metabolism, Inborn Errors; Biomarkers; Carnitine; Chromatogr

2017
Carnitine palmitoyltransferase 2 deficiency: the time-course of blood and urinary acylcarnitine levels during initial L-carnitine supplementation.
    The Tohoku journal of experimental medicine, 2010, Volume: 221, Issue:3

    Topics: Acetylcarnitine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Blood Chemical Analysis; Carniti

2010
Metabolomics identifies perturbations in human disorders of propionate metabolism.
    Clinical chemistry, 2007, Volume: 53, Issue:12

    Topics: Acetylcarnitine; Adult; Amino Acid Metabolism, Inborn Errors; Betaine; Biomarkers; Carnitine; Child;

2007
Metabolic response to carnitine in methylmalonic aciduria. An effective strategy for elimination of propionyl groups.
    Archives of disease in childhood, 1983, Volume: 58, Issue:11

    Topics: Acetylcarnitine; Amino Acid Metabolism, Inborn Errors; Carnitine; Female; Humans; Infant; Malonates;

1983
Intravenous L-carnitine and acetyl-L-carnitine in medium-chain acyl-coenzyme A dehydrogenase deficiency and isovaleric acidemia.
    Pediatric research, 1994, Volume: 35, Issue:1

    Topics: Acetylcarnitine; Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Amino Acid Metabolism, Inborn Erro

1994
Determination of acylcarnitines in urine of patients with inborn errors of metabolism using high-performance liquid chromatography after derivatization with 4'-bromophenacylbromide.
    Clinica chimica acta; international journal of clinical chemistry, 1993, Jul-16, Volume: 216, Issue:1-2

    Topics: Acetophenones; Acetylcarnitine; Acyl-CoA Dehydrogenases; Amino Acid Metabolism, Inborn Errors; Carni

1993
Prenatal diagnosis of organic acidemias based on amniotic fluid levels of acylcarnitines.
    Pediatric research, 1996, Volume: 39, Issue:4 Pt 1

    Topics: Acetylcarnitine; Acidosis; Amino Acid Metabolism, Inborn Errors; Amniotic Fluid; Female; Fetal Disea

1996
Effect of carnitine administration on glycine metabolism in patients with isovaleric acidemia: significance of acetylcarnitine determination to estimate the proper carnitine dose.
    The Tohoku journal of experimental medicine, 1996, Volume: 179, Issue:2

    Topics: Acetylcarnitine; Administration, Oral; Amino Acid Metabolism, Inborn Errors; Biomarkers; Carnitine;

1996
Restoration of hepatic cytochrome c oxidase activity and expression with acetyl-L-carnitine treatment in spf mice with an ornithine transcarbamylase deficiency.
    Biochemical pharmacology, 1998, Jun-01, Volume: 55, Issue:11

    Topics: Acetylcarnitine; Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Carbamoyl-Phosphate Synthas

1998
Reduction in the MK-801 binding sites of the NMDA sub-type of glutamate receptor in a mouse model of congenital hyperammonemia: prevention by acetyl-L-carnitine.
    Neuropharmacology, 1999, Volume: 38, Issue:3

    Topics: Acetylcarnitine; Adenosine Triphosphate; Aging; Amino Acid Metabolism, Inborn Errors; Ammonia; Anima

1999
Alteration of urinary carnitine profile induced by benzoate administration.
    Archives of disease in childhood, 1991, Volume: 66, Issue:7

    Topics: Acetylcarnitine; Administration, Oral; Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Ben

1991
Amniotic fluid propionylcarnitine in methylmalonic aciduria.
    Journal of inherited metabolic disease, 1987, Volume: 10, Issue:4

    Topics: Acetylcarnitine; Amino Acid Metabolism, Inborn Errors; Amniotic Fluid; Carnitine; Chromatography, Hi

1987
Identification of glutarylcarnitine in glutaric aciduria type 1 by carboxylic acid analyzer with an ODS reverse-phase column.
    Clinica chimica acta; international journal of clinical chemistry, 1987, May-15, Volume: 164, Issue:3

    Topics: Acetylcarnitine; Amino Acid Metabolism, Inborn Errors; Autoanalysis; Carboxylic Acids; Carnitine; Ch

1987