JCI - Citations to L-carnitine enhances excretion of propionyl coenzyme A as propionylcarnitine in propionic acidemia.

Citations to this article

L-carnitine enhances excretion of propionyl coenzyme A as propionylcarnitine in propionic acidemia.

C R Roe, … , T P Bohan, C L Hoppel

C R Roe, … , T P Bohan, C L Hoppel

Published June 1, 1984
Citation Information: J Clin Invest. 1984;73(6):1785-1788. https://doi.org/10.1172/JCI111387.

View: Text | PDF

Research Article

Total citations by year

Year:	2024	2023	2021	2020	2019	2018	2017	2016	2015	2014	2013	2012	2011	2010	2009	2008	2007	2006	2004	2003	2002	1999	1998	1996	1995	1994	1993	1992	1991	1990	1989	1988	1987	1986	1985	1984	Total
Citations:	1	2	2	2	4	3	5	4	2	2	1	2	6	2	4	2	2	2	4	3	2	2	3	2	3	3	4	5	7	4	6	1	4	11	3	1	116

Citation information

Citations to this article (116)

Title and authors	Publication	Year
How mass spectrometry revolutionized newborn screening Millington DS		2024
Organic Aciduria Disorders in Pregnancy: An Overview of Metabolic Considerations. Shakerdi LA, Gillman B, Corcoran E, McNulty J, Treacy EP	Metabolites	2023
Pathophysiological mechanisms of complications associated with propionic acidemia. Marchuk H, Wang Y, Ladd ZA, Chen X, Zhang GF	Pharmacology & Therapeutics	2023
Propionate and Alzheimer’s Disease J Killingsworth, D Sawmiller, RD Shytle	Frontiers in aging neuroscience	2021
Long-term effectiveness of carglumic acid in patients with propionic acidemia (PA) and methylmalonic acidemia (MMA): a randomized clinical trial M Alfadhel, M Nashabat, M Saleh, M Elamin, A Alfares, AA Othaim, M Umair, H Ahmed, F Ababneh, FA Mutairi, W Eyaid, A Alswaid, L Alohali, E Faqeih, M Almannai, M Aljeraisy, B Albdah, MA Hussein, Z Rahbeeni, A Alasmari	Orphanet Journal of Rare Diseases	2021
Increased protein propionylation contributes to mitochondrial dysfunction in liver cells and fibroblasts, but not in myotubes B Lagerwaard, O Pougovkina, AF Bekebrede, H Brinke, RJ Wanders, AG Nieuwenhuizen, J Keijer, VC Boer	Journal of Inherited Metabolic Disease	2020
Established and Emerging Treatments for Patients with Inborn Errors of Metabolism MS Breilyn, MP Wasserstein	NeoReviews	2020
Pathophysiology of propionic and methylmalonic acidemias. Part 1: Complications HA Haijes, JJ Jans, SY Tas, NM VerhoevenDuif, PM Hasselt	Journal of Inherited Metabolic Disease	2019
Pathophysiology of propionic and methylmalonic acidemias. Part 2: Treatment strategies HA Haijes, PM Hasselt, JJ Jans, NM VerhoevenDuif	Journal of Inherited Metabolic Disease	2019
Inborn errors of mitochondrial acyl-coenzyme a metabolism: acyl-CoA biology meets the clinic H Yang, C Zhao, MC Tang, Y Wang, SP Wang, P Allard, A Furtos, GA Mitchell	Molecular Genetics and Metabolism	2019
Sheath-flow probe electrospray ionization (sfPESI) mass spectrometry for the rapid forensic analysis of human body fluids S Rankin-Turner, S Ninomiya, JC Reynolds, K Hiraoka	Analytical Methods	2019
The Impact of Carnitine on Dietary Fiber and Gut Bacteria Metabolism and Their Mutual Interaction in Monogastrics A Ghonimy, D Zhang, M Farouk, Q Wang	International journal of molecular sciences	2018
Propionate-induced changes in cardiac metabolism, notably CoA trapping, are not altered by L-carnitine Y Wang, BA Christopher, KA Wilson, D Muoio, RW McGarrah, H Brunengraber, GF Zhang	American journal of physiology. Endocrinology and metabolism	2018
Mitochondrial Dysfunction Caused by Drugs and Environmental Toxicants Y Will, JA Dykens		2018
l-Carnitine and Acetyl-l-carnitine Roles and Neuroprotection in Developing Brain GC Ferreira, MC McKenna	Neurochemical Research	2017
Propionyl-CoA carboxylase – A review P Wongkittichote, NA Mew, KA Chapman	Molecular Genetics and Metabolism	2017
Equine atypical myopathy in the UK: Epidemiological characteristics of cases reported from 2011 to 2015 and factors associated with survival S González-Medina, JL Ireland, RJ Piercy, JR Newton, DM Votion	Equine Veterinary Journal	2017
Correcting false positive medium-chain acyl-CoA dehydrogenase deficiency results from newborn screening; synthesis, purification, and standardization of branched-chain C8 acylcarnitines for use in their selective and accurate absolute quantitation by UHPLC–MS/MS PE Minkler, MS Stoll, ST Ingalls, CL Hoppel	Molecular Genetics and Metabolism	2017
Selective and accurate C5 acylcarnitine quantitation by UHPLC–MS/MS: Distinguishing true isovaleric acidemia from pivalate derived interference PE Minkler, MS Stoll, ST Ingalls, CL Hoppel	Journal of chromatography. B, Analytical technologies in the biomedical and life sciences	2017
Impaired Exercise Performance and Skeletal Muscle Mitochondrial Function in Rats with Secondary Carnitine Deficiency J Bouitbir, P Haegler, F Singh, L Joerin, A Felser, U Duthaler, S Krähenbühl	Frontiers in physiology	2016
Disorders of branched chain amino acid metabolism I Manoli, CP Venditti	Translational Science of Rare Diseases	2016
Methylmalonic and propionic acidemias: clinical management update JL Fraser, CP Venditti	Current Opinion in Pediatrics	2016
Inborn Metabolic Diseases JM Saudubray, MR Baumgartner, J Walter	Inborn Metabolic Diseases	2016
The Encyclopedia of Mass Spectrometry SP Markey	The Encyclopedia of Mass Spectrometry	2015
Contractile function and energy metabolism of skeletal muscle in rats with secondary carnitine deficiency PA Roberts, J Bouitbir, A Bonifacio, F Singh, P Kaufmann, A Urwyler, S Krähenbühl	American journal of physiology. Endocrinology and metabolism	2015
Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia MR Baumgartner, F Hörster, M Assoun, D Ballhausen, A Burlina, KA Chapman, C Dionisi-Vici, B Fowler, S Grünert, S Grünewald, G Haliloglu, M Hochuli, T Honzik, M Huemer, D Karall, A MacDonald, D Martinelli, B Merinero, C Pérez-Cerdá, JO Sass, S Scholl-Bürgi, F Skovby, V Valayannopoulos, F Wijburg, A Chakrapani	Orphanet Journal of Rare Diseases	2014
Comprehensive Guide to Autism VB Patel, VR Preedy, CR Martin		2014
Carnitine and acylcarnitine profiles in dried blood spots of patients with acute myocardial infarction HA Khan, AS Alhomida, HA Madani, SH Sobki	Metabolomics	2013
Translating the basic knowledge of mitochondrial functions to metabolic therapy: role of L-carnitine SM Marcovina, C Sirtori, A Peracino, M Gheorghiade, P Borum, G Remuzzi, H Ardehali	Translational Research	2012
Short-chain fatty acid fermentation products of the gut microbiome: implications in autism spectrum disorders DF MacFabe	Microbial Ecology in Health and Disease	2012
Effects of the enteric bacterial metabolic product propionic acid on object-directed behavior, social behavior, cognition, and neuroinflammation in adolescent rats: Relevance to autism spectrum disorder DF MacFabe, NE Cain, F Boon, KP Ossenkopp, DP Cain	Behavioural Brain Research	2011
Profiling of acylcarnitines and sterols from dried blood or plasma spot by atmospheric pressure thermal desorption chemical ionization (APTDCI) tandem mass spectrometry G Corso, O D'Apolito, D Garofalo, G Paglia, AD Russo	Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids	2011
Acute management of propionic acidemia KA Chapman, A Gropman, E MacLeod, K Stagni, ML Summar, K Ueda, NA Mew, J Franks, E Island, D Matern, L Pena, B Smith, VR Sutton, T Urv, C Venditti, A Chakrapani	Molecular Genetics and Metabolism	2011
Chronic management and health supervision of individuals with propionic acidemia VR Sutton, KA Chapman, AL Gropman, E MacLeod, K Stagni, ML Summar, K Ueda, NA Mew, J Franks, E Island, D Matern, L Peña, B Smith, T Urv, C Vendetti, A Chakarapani	Molecular Genetics and Metabolism	2011
Adeno-Associated Virus Serotype 8 Gene Transfer Rescues a Neonatal Lethal Murine Model of Propionic Acidemia RJ Chandler, S Chandrasekaran, N Carrillo-Carrasco, JS Senac, SE Hofherr, MA Barry, CP Venditti	Human Gene Therapy	2011
On the protective effect of omega-3 against propionic acid-induced neurotoxicity in rat pups AK El-Ansary, SK Al-Daihan, AR El-Gezeery	Lipids in Health and Disease	2011
Pädiatrie Karall D, Meisinger B, Grissenauer G, Scholl-Bürgi S, Heinz-Erian P, Lagler F, Sass JO, Grünert S, Nussbaumer EM, Schwab KO, Mönch E, Hofer A, Haberlandt E, Oppl V, Sperl W, Spiekerkötter U, Baumgartner Sigl S, Stöckler-Ipsiroglu S		2010
Altered brain phospholipid and acylcarnitine profiles in propionic acid infused rodents: further development of a potential model of autism spectrum disorders RH Thomas, KA Foley, JR Mepham, LJ Tichenoff, F Possmayer, DF MacFabe	Journal of Neurochemistry	2010
Short-Term Rescue of Neonatal Lethality in a Mouse Model of Propionic Acidemia by Gene Therapy SE Hofherr, JS Senac, CY Chen, DJ Palmer, P Ng, MA Barry	Human Gene Therapy	2009
Mass spectrometry in newborn and metabolic screening: historical perspective and future directions DH Chace	Journal of Mass Spectrometry	2009
Early nerve ending rescue from oxidative damage and energy failure by l-carnitine as post-treatment in two neurotoxic models in rat: recovery of antioxidant and reductive capacities D Elinos-Calderón, Y Robledo-Arratia, VP la Cruz, J Pedraza-Chaverrí, SF Ali, A Santamaría	Experimental Brain Research	2009
Early nerve ending rescue from oxidative damage and energy failure by l-carnitine as post-treatment in two neurotoxic models in rat: recovery of antioxidant and reductive capacities D Elinos-Calderón, Y Robledo-Arratia, VP Cruz, J Pedraza-Chaverrí, SF Ali, A Santamaría	Experimental Brain Research	2009
Hereditary and acquired diseases of acyl-coenzyme A metabolism GA Mitchell, N Gauthier, A Lesimple, SP Wang, O Mamer, I Qureshi	Molecular Genetics and Metabolism	2008
Laboratory Guide to the Methods in Biochemical Genetics N Blau, M Duran, KM Gibson		2008
Development of a newborn screening follow-up algorithm for the diagnosis of isobutyryl-CoA dehydrogenase deficiency D Oglesbee, M He, N Majumder, J Vockley, A Ahmad, B Angle, B Burton, J Charrow, R Ensenauer, CH Ficicioglu, LD Keppen, D Marsden, S Tortorelli, SH Hahn, D Matern	Genetics in Medicine	2007
Two Step Derivatization for the Analyses of Organic, Amino Acids and Glycines on Filter Paper Plasma by GC-MS/SIM HR Yoon	Archives of Pharmacal Research	2007
Diagnosis and early management of inborn errors of metabolism presenting around the time of birth JV Leonard, AA Morris	Acta Paediatrica	2006
Diagnosis and early management of inborn errors of metabolism presenting around the time of birth1 J Leonard, A Morris	Acta paediatrica (Oslo, Norway : 1992)	2006
l-Carnitine Is a Modulator of the Glucocorticoid Receptor Alpha S Alesci, MU Martino, T Kino, I Ilias	Annals of the New York Academy of Sciences	2004
Neuroprotective effect of l-carnitine in the 3-nitropropionic acid (3-NPA)-evoked neurotoxicity in rats Z Binienda	Neuroscience Letters	2004
Therapeutic Effects of l-Carnitine and Propionyl-l-carnitine on Cardiovascular Diseases: A Review R Ferrari, E Merli, G Cicchitelli, D Mele, A Fucili, C Ceconi	Annals of the New York Academy of Sciences	2004
Carnitine Deficiency Disorders in Children CA Stanley	Annals of the New York Academy of Sciences	2004
L-Carnitine in inborn errors of metabolism: What is the evidence? JH Walter	Journal of Inherited Metabolic Disease	2003
Use of Tandem Mass Spectrometry for Multianalyte Screening of Dried Blood Specimens from Newborns DH Chace, TA Kalas, EW Naylor	Clinical chemistry	2003
l ‐Carnitine: a nutritional modulator of glucocorticoid receptor functions S Alesci, MU Martino, M Mirani, S Benvenga, F Trimarchi, T Kino, GP Chrousos	The FASEB Journal	2003
Newer components of enteral formulas T Sentongo, MR Mascarenhas	Pediatric Clinics of North America	2002
Emergency management of inherited metabolic diseases V Prietsch, M Lindner, J Zschocke, WL Nyhan, GF Hoffmann	Journal of Inherited Metabolic Disease	2002
18Fluoro-2-deoxyglucose (18FDG) PET scan of the brain in propionic acidemia: clinical and MRI correlations M Al-Essa	Brain and Development	1999
Formation of Propionate After Short-Term Ethanol Treatment and Its Interaction with the Carnitine Pool in Rat V Calabrese, V Rizza	Alcohol	1999
Infectious complications of propionic acidemia in Saudia Arabia MA Essa, Z Rahbeeni, S Jumaah, S Joshi, EA Jishi, MS Rashed, MA Amoudi, PT Ozand	Clinical Genetics	1998
Methylmalonic Semialdehyde Dehydrogenase Deficiency: Psychomotor Delay and Methylmalonic Aciduria without Metabolic Decompensation CR Roe, E Struys, RM Kok, DS Roe, RA Harris, C Jakobs	Molecular Genetics and Metabolism	1998
Carnitine deficiency-induced cardiomyopathy D J Paulson	Molecular and Cellular Biochemistry	1998
Review M Epstein	Renal Failure	1996
Inhibition of carnitine biosynthesis by valproic acid in rats—The biochemical mechanism of inhibition V Farkas	Biochemical Pharmacology	1996
Acylcarnitines in intermediary metabolism AC Sewell, HJ Böhles	European Journal of Pediatrics	1995
On the differences between urinary metabolite excretion and odd-numbered fatty acid production in propionic and methylmalonic acidaemias U Wendel, A Eißler, W Sperl, P Schadewaldt	Journal of Inherited Metabolic Disease	1995
The management and outcome of propionic and methylmalonic acidaemia JV Leonard	Journal of Inherited Metabolic Disease	1995
Carnitine esters in metabolic disease H Böhles, A Evangeliou, K Bervoets, I Eckert, A Sewell	European Journal of Pediatrics	1994
Hagers Handbuch der Pharmazeutischen Praxis M Albinus, G Amschler, U Amschler, E von Angerer, W Barthel, A Bauer, K Bauer, J Beckmann, W Beil, J Beitz, P Berscht, T Beyrich, K Binder, R Böhme, HH Borchert, F Bracher, H Bräunlich, K Brinkmann, F von Bruchhausen, K Brune, A Büge, W Christ, M Cimbollek, R Daniels, G Dannhardt, HJ Duchstein, S Ebel, K Eger, P Emig, T Erker, MS Fernandez-Alfonso, AW Frahm, M Frahm, KJ Freundt, D Geffken, U Geis, E Glusa, B Göber, J Gödicke, M Goppelt-Strübe, W Gössling, A Graul, S Greiner, A Grisk, M Grosam, M von Gruchalla, M Gütschow, A Haberkorn, E Hackenthal, A Häfner, B Haluszczynski, A Harder, S Hartmann, S Hartmann, D Heber, G Heinemeyer, A Hensel, S Hoedt-Schmidt, H Hoffmann, U Hoffmann-Schollmayer, U Holzgrabe, M Hug, W Ibrom, E Inkmann, J Jürgens, B Kaiser, DA Kalbhen, N Khudeir, W Kiefer, D Kleinsorge, C Klett, S Klett, M Klingmüller, HP Klöcking, G Kobal, A Kramer, M Kreher, G Kreutz, R Kroker, D Landsiedel-Maier, J Lehmann, MS Lehner, S Leiner, H Löwe, J Mann, D Manns, W Matthiessen, A Maurer, W Meindl, K Menges, P Messinger, HJ Mest, FP Meyer, J Mollière, R Morgenstern, W Morick, J Mössner, C Müller, D Müller, K Müller, W Müller, A Mülsch, T Netzer, M Neugebauer, U Niemeyer, P Nuhn, B Nürnberg, J Oertel, M Oettel, H Oßwald, R Ott, T Ott, T Otzen, P Pachaly, H Pelzer, KU Petersen, M Pickert, A Pies, K Pietrzik, D Preiss, H Priewer, O Queckenberg, E Reimann, J Remien, R Rettig, A Reymann, J Richter, H Rommelspacher, U Rose, G Rücker, K Schaefer, H Schlager, W Schleicher, H Schleinitz, W Schlichter, H Schmidhammer, G Schmidt, T Schmidt, J Schräder, T Schulz, G Schulze, P Schwanz, H Schwilden, M Serke, G Skopp, M Spohn, R Stahlmann, JP Stasch, C Steffen, J Steinmeyer, KH Surborg, U Sürig, I Szelenyi, R Troschütz, M Verborg, EJ Verspohl, FJ Volk, H Walther, H Weber, J Wede, M Wenzel, M Weyandt-Spangenberg, S Wich, H Winterhoff, R Wintersteiger, F Woltmann, D Youssef, A Ziegler		1994
Urinary propionylcarnitine analysis for monitoring carnitine supplementation in inherited disorders of propionate metabolism N Sugiyama, I Matsuda, Y Wada, K Narisawa, M Kobayashi, K Kidouchi	Journal of Inherited Metabolic Disease	1994
Factors affecting the distribution of ingested propionic acid in the rat forestomach JE Bueld, KJ Netter	Food and Chemical Toxicology	1993
Effect of treatment with glycine and l-carnitine in medium-chain acyl-coenzyme A dehydrogenase deficiency P Rinaldo, E Schmidt-Sommerfeld, AP Posca, SJ Heales, DA Woolf, JV Leonard	The Journal of Pediatrics	1993
Propionate regulates lymphocyte proliferation and metabolism R Curi, JA Bond, PC Calder, EA Newsholme	General Pharmacology: The Vascular System	1993
Acylcarnitines in amniotic fluid: Application to the prenatal diagnosis of propionic acidaemia JL Hove, DH Chace, SG Kahler, DS Millington	Journal of Inherited Metabolic Disease	1993
Applications of isotope dilution-mass spectrometry in clinical chemistry, pharmacokinetics, and toxicology AP de Leenheer, LM Thienpont	Mass Spectrometry Reviews	1992
Case reports of successful pregnancy in women with maple syrup urine disease and propionic acidemia SC van Calcar, CO Harding, SR Davidson, LA Barness, JA Wolff	American Journal of Medical Genetics	1992
Hepatic Encephalopathy JE Maddison	Journal of Veterinary Internal Medicine	1992
Prevalence of carnitine depletion in critically ill patients with undernutrition A Wennberg, A Hyltander, Å Sjöberg, B Arfvidsson, R Sandström, I Wickström, K Lundholm	Metabolism	1992
Plasma carnitine in fasting neonatal and adult northern elephant seals SH Adams, DP Costa, SC Winter	American journal of physiology. Endocrinology and metabolism	1992
Inadequate iron availability as a possible cause of low serum carnitine concentrations in patients with phenylketonuria H Böhles, K Ullrich, W Endres, AW Behbehani, U Wendel	European Journal of Pediatrics	1991
Quantitative estimation of absorption and degradation of a carnitine supplement by human adults CJ Rebouche	Metabolism	1991
Propionate mitochondrial toxicity in liver and skeletal muscle: Acyl CoA levels T Matsuishi, DA Stumpf, M Seliem, LA Eguren, K Chrislip	Biochemical Medicine and Metabolic Biology	1991
Long-term l-carnitine treatment in isovaleric acidemia E Mayatepek, TW Kurczynski, CL Hoppel	Pediatric Neurology	1991
Effect of exogenous carnitine on carnitine homeostasis in the rat LJ Ruff, LG Miller, EP Brass	Biochimica et Biophysica Acta (BBA) - General Subjects	1991
Topical Review Article: Organic Acidurias: A Review Part 2 PT Ozand, GG Gascon	Journal of child neurology	1991
Progressive renal insufficiency in methylmalonic acidemia KH Molteni, TD Oberley, JA Wolff, AL Friedman	Pediatric Nephrology	1991
Carnitine deficiency SC Winter, EM Zorn, WH Vance, RA Chalmers, MD Bain, TE Stacey, CD Sousa	The Lancet	1990
The effect of L-carnitine supplementation in 4 pentenoic acid treated rats T Sugimoto, M Woo, N Nishida, A Araki, K Murakami, Y Kobayashi	Brain and Development	1990
Rapid Spectrophotometric Determination of Plasma Carnitine Concentrations J Maeda, SJ Dudrick	JPEN. Journal of parenteral and enteral nutrition	1990
Cirrhosis, Hepatic Encephalopathy, and Ammonium Toxicity S Grisolía, V Felipo, MD Miñana		1990
Medium-chain acyl-CoA dehydrogenase deficiency: Metabolic effects and therapeutic efficacy of long-terml-carnitine supplementation WR Treem, CA Stanley, SI Goodman	Journal of Inherited Metabolic Disease	1989
Measurement of urinary free and acylcarnitines: Quantitative acylcarnitine profiling in normal humans and in several patients with metabolic errors JA Montgomery, OA Mamer	Analytical Biochemistry	1989
Substrate disposal in metabolic disease: A comparison between rates of in vivo propionate oxidation and urinary metabolite excretion in children with methylmalonic acidemia GN Thompson, JH Walter, JL Bresson, GC Ford, JP Bonnefont, RA Chalmers, JM Saudubray, JV Leonard, D Halliday	The Journal of Pediatrics	1989
Parenteral nutrition in propionic and methylmalonic acidemia SG Kahler, DS Millington, SD Cederbaum, J Vargas, LD Bond, DA Maltby, DS Gale, CR Roe	The Journal of Pediatrics	1989
Application of fast atom bombardment with tandem mass spectrometry and liquid chromatography/mass spectrometry to the analysis of acylcarnitines in human urine, blood, and tissue DS Millington, DL Norwood, N Kodo, CR Roe, F Inoue	Analytical Biochemistry	1989
Hypoglycemia in Infants and Children MW Haymond	Endocrinology and metabolism clinics of North America	1989
Effects of propionate and carnitine on the hepatic oxidation of short- and medium-chain-length fatty acids EP Brass, RA Beyerinck	Biochemical Journal	1988
Characterization of new diagnostic acylcarnitines in patients with β-ketothiolase deficiency and glutaric aciduria type I using mass spectrometry DS Millington, CR Roe, DA Maltby	Biological Mass Spectrometry	1987
High-performance liquid chromatography of coenzyme A esters formed by transesterification of short-chain acylcarnitines: Diagnosis of acidemias by urinary analysis RE Dugan, MJ Schmidt, GE Hoganson, J Steele, BA Gilles, AL Shug	Analytical Biochemistry	1987
Identification of glutarylcarnitine in glutaric aciduria type 1 by carboxylic acid analyzer with an ODS reverse-phase column K Kidouchi, N Sugiyama, H Morishita, M Kobayashi, Y Wada, D Nohara	Clinica Chimica Acta	1987
l-Carnitine: A Preliminary Review of its Pharmacokinetics, and its Therapeutic Use in Ischaemic Cardiac Disease and Primary and Secondary Carnitine Deficiencies in Relationship to its Role in Fatty Acid Metabolism KL Goa, RN Brogden	Drugs	1987
Identification of 3-methylglutarylcarnitine. A new diagnostic metabolite of 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency CR Roe, DS Millington, DA Maltby	Journal of Clinical Investigation	1986
Direct identification of propionylcarnitine in propionic acidaemia: Biochemical and clinical results of oral carnitine supplementation M Duran, D Ketting, TE Beckeringh, D Leupold, SK Wadman	Journal of Inherited Metabolic Disease	1986
Effects of organic acids on the synthesis of citrulline by intact rat liver mitochondria D Rabier, P Briand, F Petit, P Kamoun, L Cathelineau	Biochimie	1986
CARNITINE REDUCES FASTING KETOGENESIS IN PATIENTS WITH DISORDERS OF PROPIONATE METABOLISM JA Wolff, LP Thuy, R Haas, JE Carroll, C Prodanos, WL Nyhan	The Lancet	1986
Inhibition of oxidative metabolism by propionic acid and its reversal by carnitine in isolated rat hepatocytes EP Brass, PV Fennessey, LV Miller	Biochemical Journal	1986
Serum and urinary carnitine and organic acids in reye syndrome and Reye-like syndrome T Sugimoto, N Nishida, M Woo, T Takeuchi, A Yasuhara, Y Kobayashi, Y Sakane	Brain and Development	1986
Separation of acylcarnitines from biological samples using high-performance liquid chromatography CL Hoppel, EP Brass, AP Gibbons, JS Turkaly	Analytical Biochemistry	1986
Analysis of volatile free fatty acids in human urine by capillary column gas chromatography/mass spectrometry DA Maltby, DS Millington	Clinica Chimica Acta	1986
Differentiation of isomeric acyl carnitines using tandem mass spectrometry SJ Gaskell, C Guenat, DS Millington, DA Maltby, CR Roe	Analytical Chemistry	1986
Rank annihilation with incomplete information DH Burns, JB Callis, GD Christian	Analytical Chemistry	1986
Quantitation of the efflux of acylcarnitines from rat heart, brain, and liver mitochondria W Lysiak, PP Toth, CH Suelter, LL Bieber	The Journal of biological chemistry	1986
Plasma and muscle free carnitine deficiency due to renal Fanconi syndrome I Bernardini, WB Rizzo, M Dalakas, J Bernar, WA Gahl	Journal of Clinical Investigation	1985
An improved method for the determination of free and esterified carnitine C Rössle, KP Kohse, HE Franz, P Fürst	Clinica Chimica Acta	1985
Octanoylglucuronide excretion in patients with a defective oxidation of medium-chain fatty acids M Duran, D Ketting, R Vossen, TE Beckeringh, L Dorland, L Bruinvis, SK Wadman	Clinica Chimica Acta	1985
L-carnitine therapy in isovaleric acidemia CR Roe, DS Millington, DA Maltby, SG Kahler, TP Bohan	Journal of Clinical Investigation	1984

Advertisement