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acetylcarnitine and Lipid Metabolism, Inborn Error

acetylcarnitine has been researched along with Lipid Metabolism, Inborn Error in 11 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
"A 47-year-old man suffering from a bipolar disorder and intermittent myoglobinuria presented with acute rhabdomyolysis with renal failure after starting therapy with valproic acid."7.71Valproic acid triggers acute rhabdomyolysis in a patient with carnitine palmitoyltransferase type II deficiency. ( Glocker, FX; Jaksch, M; Ketelsen, UP; Kottlors, M; Lücking, CH; Weiner, S, 2001)
"A 47-year-old man suffering from a bipolar disorder and intermittent myoglobinuria presented with acute rhabdomyolysis with renal failure after starting therapy with valproic acid."3.71Valproic acid triggers acute rhabdomyolysis in a patient with carnitine palmitoyltransferase type II deficiency. ( Glocker, FX; Jaksch, M; Ketelsen, UP; Kottlors, M; Lücking, CH; Weiner, S, 2001)
"Rhabdomyolysis is common in very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) and other metabolic myopathies, but its pathogenic basis is poorly understood."1.43Altered Energetics of Exercise Explain Risk of Rhabdomyolysis in Very Long-Chain Acyl-CoA Dehydrogenase Deficiency. ( de Sain-van der Velden, M; Diekman, EF; Houten, SM; Jeneson, JA; Nievelstein, RA; Schmitz, JP; Takken, T; Van der Pol, WL; van Riel, NA; Visser, G; Wardrop, M, 2016)

Research

Studies (11)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's5 (45.45)18.2507
2000's3 (27.27)29.6817
2010's3 (27.27)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Diekman, EF1
Visser, G1
Schmitz, JP1
Nievelstein, RA1
de Sain-van der Velden, M1
Wardrop, M1
Van der Pol, WL1
Houten, SM1
van Riel, NA1
Takken, T1
Jeneson, JA1
Hori, T1
Fukao, T1
Kobayashi, H1
Teramoto, T1
Takayanagi, M1
Hasegawa, Y1
Yasuno, T1
Yamaguchi, S2
Kondo, N1
Behrend, AM1
Harding, CO1
Shoemaker, JD1
Matern, D2
Sahn, DJ1
Elliot, DL1
Gillingham, MB1
Iacobazzi, V1
Pasquali, M1
Singh, R1
Rinaldo, P1
Amat di San Filippo, C1
Palmieri, F1
Longo, N1
Van Hove, JL1
Kahler, SG1
Millington, DS1
Roe, DS1
Chace, DH1
Heales, SJ1
Roe, CR1
Poorthuis, BJ1
Jille-Vlcková, T1
Onkenhout, W1
Horiuchi, M1
Kobayashi, K1
Asaka, N1
Saheki, T1
Kimura, M1
al Aqeel, AI1
Rashed, MS1
Wanders, RJ1
Kottlors, M1
Jaksch, M1
Ketelsen, UP1
Weiner, S1
Glocker, FX1
Lücking, CH1
Invernizzi, F1
Burlina, AB1
Donadio, A1
Giordano, G1
Taroni, F1
Garavaglia, B1

Clinical Trials (4)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Acute Nutritional Ketosis and Exercise in Glycogen Storage Disease Type IIIa[NCT03011203]6 participants (Actual)Interventional2017-02-10Completed
Acute Nutritional Ketosis in VLCAD Deficiency: Testing the Metabolic Base for Therapeutic Use[NCT03531554]5 participants (Actual)Interventional2016-04-01Completed
Fatty Acid Oxidation Disorders & Body Weight Regulation[NCT00654004]26 participants (Actual)Observational2006-04-30Completed
Effect of the Administration of L-Carnitine on Body Fat Percentage and Body Weight in Overweight Women in Crossfit Training.[NCT03436277]Phase 224 participants (Anticipated)Interventional2018-12-17Not yet recruiting
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

An Outcome of This Study is the Difference in Glucose Tolerance Between Subjects With a Long-chain Fatty Acid Oxidation Disorder and Normal Controls.

"Glucose tolerance was estimated by the Matsuda Index using glucose and insulin values from a standard oral glucose tolerance test. The Matsuda Index is calculated by the following formula: 10,000/ sq root of (fasting glucose mg/dl X fasting insulin in units/ml) X (mean glucose (mg/dl) X mean insulin (units/ml) and correlates with insulin sensitivity measured by the gold standard method of a hyperinsulinemic euglycemic clamp. Values of 2.5 or greater are considered insulin sensitive. Values of 2.4 or less are considered insulin resistance.~The Matsuda Index of Insulin Sensitivity was measured in subjects with a long-chain fatty acid oxidation disorder (n=12). Twelve age, sex and BMI matched controls and 4 heterozygotes for a long-chain fatty acid oxidation disorder were recruited who also completed an oral glucose tolerance test. The difference in Mastuda Index between subjects and age matched controls was compared by t-test." (NCT00654004)
Timeframe: Subjects will be compared to controls at one point in time.

Interventionunits on a scale (Mean)
Subjects2.8
Controls3.13

An Outcome of This Study is the Difference in Percent Body Fat (%BF) Between Subjects With a Long-chain Fatty Acid Oxidation Disorder and Normal Controls.

Body composition by DEXA was measured in subjects with a long-chain fatty acid oxidation disorder (n=13). Twelve age, sex and BMI matched controls and 4 heterozygotes for a long-chain fatty acid oxidation disorder were recruited who also completed body composition measures. The difference in body composition between subjects and age matched controls was compared by t-test. (NCT00654004)
Timeframe: Subjects will be compared to controls at one point in time.

Interventionpercentage of body fat (Mean)
Subjects31.8
Controls27.2

The Difference in Plasma Adiponectin Levels Between Subjects With a Long-chain Fatty Acid Oxidation Disorder and Matched Controls Was Compared by T-test

Fasting total adiponectin levels in ug/ml were measured in both groups (subjects with a long-chain fatty acid oxidation disorder). The differences between groups were compared with a t-test (NCT00654004)
Timeframe: Fasting total adiponectin (ug/ml)

Interventionug/ml (Mean)
Subjects17.3
Controls24.4

The Difference in Plasma Insulin Between Subjects With a Long-chain Fatty Acid Oxidation Disorder and Matched Controls Was Compared by T-test

Fasting insulin levels in uU/ml were measured in both groups. The differences between groups were compared with a t-test (NCT00654004)
Timeframe: Fasting insulin levels uUnits/ml

InterventionuU/ml (Mean)
Subjects17
Controls13

The Difference in Plasma Leptin Between Subjects With a Long-chain Fatty Acid Oxidation Disorder and Matched Controls Was Compared by T-test

Fasting leptin in ng/kg fat mass were measured in both groups (subjects with a long-chain fatty acid oxidation disorder; controls). The differences between groups were compared with a t-test (NCT00654004)
Timeframe: Fasting leptin levels ng per kg of fat mass

Interventionng/kg (Mean)
Subjects0.99
Controls0.91

Reviews

1 review available for acetylcarnitine and Lipid Metabolism, Inborn Error

ArticleYear
[2,4-Dienoyl-CoA reductase deficiency].
    Ryoikibetsu shokogun shirizu, 1998, Issue:18 Pt 1

    Topics: Acetylcarnitine; Biomarkers; Diagnosis, Differential; Fatty Acid Desaturases; Humans; Lipid Metaboli

1998

Trials

1 trial available for acetylcarnitine and Lipid Metabolism, Inborn Error

ArticleYear
Substrate oxidation and cardiac performance during exercise in disorders of long chain fatty acid oxidation.
    Molecular genetics and metabolism, 2012, Volume: 105, Issue:1

    Topics: Acetylcarnitine; Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Child; Creatine Kinase; Demo

2012
Substrate oxidation and cardiac performance during exercise in disorders of long chain fatty acid oxidation.
    Molecular genetics and metabolism, 2012, Volume: 105, Issue:1

    Topics: Acetylcarnitine; Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Child; Creatine Kinase; Demo

2012
Substrate oxidation and cardiac performance during exercise in disorders of long chain fatty acid oxidation.
    Molecular genetics and metabolism, 2012, Volume: 105, Issue:1

    Topics: Acetylcarnitine; Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Child; Creatine Kinase; Demo

2012
Substrate oxidation and cardiac performance during exercise in disorders of long chain fatty acid oxidation.
    Molecular genetics and metabolism, 2012, Volume: 105, Issue:1

    Topics: Acetylcarnitine; Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Child; Creatine Kinase; Demo

2012

Other Studies

9 other studies available for acetylcarnitine and Lipid Metabolism, Inborn Error

ArticleYear
Altered Energetics of Exercise Explain Risk of Rhabdomyolysis in Very Long-Chain Acyl-CoA Dehydrogenase Deficiency.
    PloS one, 2016, Volume: 11, Issue:2

    Topics: Acetylcarnitine; Acyl-CoA Dehydrogenase, Long-Chain; Adenosine Triphosphate; Adolescent; Adult; Case

2016
Altered Energetics of Exercise Explain Risk of Rhabdomyolysis in Very Long-Chain Acyl-CoA Dehydrogenase Deficiency.
    PloS one, 2016, Volume: 11, Issue:2

    Topics: Acetylcarnitine; Acyl-CoA Dehydrogenase, Long-Chain; Adenosine Triphosphate; Adolescent; Adult; Case

2016
Altered Energetics of Exercise Explain Risk of Rhabdomyolysis in Very Long-Chain Acyl-CoA Dehydrogenase Deficiency.
    PloS one, 2016, Volume: 11, Issue:2

    Topics: Acetylcarnitine; Acyl-CoA Dehydrogenase, Long-Chain; Adenosine Triphosphate; Adolescent; Adult; Case

2016
Altered Energetics of Exercise Explain Risk of Rhabdomyolysis in Very Long-Chain Acyl-CoA Dehydrogenase Deficiency.
    PloS one, 2016, Volume: 11, Issue:2

    Topics: Acetylcarnitine; Acyl-CoA Dehydrogenase, Long-Chain; Adenosine Triphosphate; Adolescent; Adult; Case

2016
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
Response to therapy in carnitine/acylcarnitine translocase (CACT) deficiency due to a novel missense mutation.
    American journal of medical genetics. Part A, 2004, Apr-15, Volume: 126A, Issue:2

    Topics: Acetylcarnitine; Carnitine; Carnitine Acyltransferases; Child, Preschool; Consanguinity; Dicarboxyli

2004
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
Secondary abnormality of carnitine biosynthesis results from carnitine reabsorptional system defect in juvenile visceral steatosis mice.
    Biochimica et biophysica acta, 1997, Dec-31, Volume: 1362, Issue:2-3

    Topics: Acetylcarnitine; Animals; Betaine; Biological Transport; Carnitine; Kidney; Lipid Metabolism, Inborn

1997
Carnitine-acylcarnitine translocase deficiency is a treatable disease.
    Journal of inherited metabolic disease, 1999, Volume: 22, Issue:3

    Topics: Acetylcarnitine; Carnitine; Carnitine Acyltransferases; Female; Humans; Infant, Newborn; Lipid Metab

1999
Valproic acid triggers acute rhabdomyolysis in a patient with carnitine palmitoyltransferase type II deficiency.
    Neuromuscular disorders : NMD, 2001, Volume: 11, Issue:8

    Topics: Acetylcarnitine; Acute Disease; Antimanic Agents; Bipolar Disorder; Carnitine O-Palmitoyltransferase

2001
Lethal neonatal presentation of carnitine palmitoyltransferase I deficiency.
    Journal of inherited metabolic disease, 2001, Volume: 24, Issue:5

    Topics: Acetylcarnitine; Bradycardia; Carnitine O-Palmitoyltransferase; Fatal Outcome; Heart Arrest; Humans;

2001