Arnold GL, Van Hove J, Freedenberg D, Strauss A, Longo N, Burton B, Garganta C, Ficicioglu C, Cederbaum S, Harding C, Boles RG, Matern D, Chakraborty P, Feigenbaum A: A Delphi clinical practice protocol for the management of very long chain acyl-CoA dehydrogenase deficiency. Mol Genet Metab 2009, 96: 85–90. 10.1016/j.ymgme.2008.09.008
Article
CAS
Google Scholar
Spiekerkoetter U, Sun B, Zytkovicz T, Wanders R, Strauss AW, Wendel U: MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency. J Pediatr 2003, 143: 335–342. 10.1067/S0022-3476(03)00292-0
Article
Google Scholar
Strauss AW, Powell CK, Hale DE, Anderson MM, Ahuja A, Brackett JC, Sims HF: Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood. Proc Natl Acad Sci USA 1995, 92: 10496–10500. 10.1073/pnas.92.23.10496
Article
CAS
Google Scholar
Gregersen N, Andresen BS, Corydon MJ, Corydon TJ, Olsen RK, Bolund L, Bross P: Mutation analysis in mitochondrial fatty acid oxidation defects: exemplified by acyl-CoA dehydrogenase deficiencies, with special focus on genotype-phenotype relationship. Hum Mutat 2001, 18: 169–189. 10.1002/humu.1174
Article
CAS
Google Scholar
Kompare M, Rizzo WB: Mitochondrial fatty-acid oxidation disorders. Semin Pediatr Neurol 2008, 15: 140–149. 10.1016/j.spen.2008.05.008
Article
Google Scholar
Chegary M, Brinke H, Ruiter JP, Wijburg FA, Stoll MS, Minkler PE, van Weeghel M, Schulz H, Hoppel CL, Wanders RJ, Houten SM: Mitochondrial long chain fatty acid beta-oxidation in man and mouse. Biochim Biophys Acta 2009, 1791: 806–815. 10.1016/j.bbalip.2009.05.006
Article
CAS
Google Scholar
Exil VJ, Gardner CD, Rottman JN, Sims H, Bartelds B, Khuchua Z, Sindhal R, Ni G, Strauss AW: Abnormal mitochondrial bioenergetics and heart rate dysfunction in mice lacking very-long-chain acyl-CoA dehydrogenase. Am J Physiol Heart Circ Physiol 2006, 290: H1289–1297. 10.1152/ajpheart.00811.2005
Article
CAS
Google Scholar
Goetzman ES, Tian L, Wood PA: Differential induction of genes in liver and brown adipose tissue regulated by peroxisome proliferator-activated receptor-alpha during fasting and cold exposure in acyl-CoA dehydrogenase-deficient mice. Mol Genet Metab 2005, 84: 39–47. 10.1016/j.ymgme.2004.09.010
Article
CAS
Google Scholar
Spiekerkoetter U, Ruiter J, Tokunaga C, Wendel U, Mayatepek E, Wijburg FA, Strauss AW, Wanders RJ: Evidence for impaired gluconeogenesis in very long-chain acyl-CoA dehydrogenase-deficient mice. Horm Metab Res 2006, 38: 625–630. 10.1055/s-2006-954581
Article
CAS
Google Scholar
Spiekerkoetter U, Tokunaga C, Wendel U, Mayatepek E, Ijlst L, Vaz FM, van Vlies N, Overmars H, Duran M, Wijburg FA, Wanders RJ, Strauss AW: Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice. Pediatr Res 2005, 57: 760–764. 10.1203/01.PDR.0000157915.26049.47
Article
CAS
Google Scholar
Tucci S, Primassin S, Spiekerkoetter U: Fasting-induced oxidative stress in very long chain acyl-CoA dehydrogenase-deficient mice. FEBS J 2010, 277: 4699–4708. 10.1111/j.1742-4658.2010.07876.x
Article
CAS
Google Scholar
Tucci S, Primassin S, Ter Veld F, Spiekerkoetter U: Medium-chain triglycerides impair lipid metabolism and induce hepatic steatosis in very long-chain acyl-CoA dehydrogenase (VLCAD)-deficient mice. Mol Genet Metab 2010, 101: 40–47. 10.1016/j.ymgme.2010.05.005
Article
CAS
Google Scholar
Exil VJ, Roberts RL, Sims H, McLaughlin JE, Malkin RA, Gardner CD, Ni G, Rottman JN, Strauss AW: Very-long-chain acyl-coenzyme a dehydrogenase deficiency in mice. Circ Res 2003, 93: 448–455. 10.1161/01.RES.0000088786.19197.E4
Article
CAS
Google Scholar
Tucci S, Flogel U, Hermann S, Sturm M, Schafers M, Spiekerkoetter U: Development and pathomechanisms of cardiomyopathy in very long-chain acyl-CoA dehydrogenase deficient (VLCAD) mice. Biochimica et Biophysica Acta 2014, 1842(5):677–685. 10.1016/j.bbadis.2014.02.001
Article
CAS
Google Scholar
Tucci S, Herebian D, Sturm M, Seibt A, Spiekerkoetter U: Tissue-specific strategies of the very-long chain acyl-CoA dehydrogenase-deficient (VLCAD(−/−)) mouse to compensate a defective fatty acid beta-oxidation. PloS One 2012, 7: e45429. 10.1371/journal.pone.0045429
Article
CAS
Google Scholar
Tucci S, Pearson S, Herebian D, Spiekerkoetter U: Long-term dietary effects on substrate selection and muscle fiber type in very-long-chain acyl-CoA dehydrogenase deficient (VLCAD(−/−)) mice. Biochim Biophys Acta 1832, 2013: 509–516.
Google Scholar
Bradford MM: A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding. Anal Biochem 1976, 72: 248–254. 10.1016/0003-2697(76)90527-3
Article
CAS
Google Scholar
Mohanty JG, Jaffe JS, Schulman ES, Raible DG: A highly sensitive fluorescent micro-assay of H
2
O
2
release from activated human leukocytes using a dihydroxyphenoxazine derivative. J Immunol Meth 1997, 202: 133–141. 10.1016/S0022-1759(96)00244-X
Article
CAS
Google Scholar
Lawrence RA, Burk RF: Glutathione peroxidase activity in selenium-deficient rat liver. Biochem Biophys Res Comm 1976, 71: 952–958. 10.1016/0006-291X(76)90747-6
Article
CAS
Google Scholar
Mantha SV, Prasad M, Kalra J, Prasad K: Antioxidant enzymes in hypercholesterolemia and effects of vitamin E in rabbits. Atherosclerosis 1993, 101: 135–144. 10.1016/0021-9150(93)90110-G
Article
CAS
Google Scholar
Milder JB, Liang LP, Patel M: Acute oxidative stress and systemic Nrf2 activation by the ketogenic diet. Neurobiol Dis 2010, 40: 238–244. 10.1016/j.nbd.2010.05.030
Article
CAS
Google Scholar
Primassin S, Ter Veld F, Mayatepek E, Spiekerkoetter U: Carnitine supplementation induces acylcarnitine production in tissues of very long-chain acyl-CoA dehydrogenase-deficient mice, without replenishing low free carnitine. Pediatr Res 2008, 63: 632–637. 10.1203/PDR.0b013e31816ff6f0
Article
CAS
Google Scholar
Primassin S, Tucci S, Herebian D, Seibt A, Hoffmann L, ter Veld F, Spiekerkoetter U: Pre-exercise medium-chain triglyceride application prevents acylcarnitine accumulation in skeletal muscle from very-long-chain acyl-CoA-dehydrogenase-deficient mice. J Inherit Metab Dis 2010, 33: 237–246. 10.1007/s10545-010-9105-7
Article
CAS
Google Scholar
Kersten S, Seydoux J, Peters JM, Gonzalez FJ, Desvergne B, Wahli W: Peroxisome proliferator-activated receptor alpha mediates the adaptive response to fasting. J Clin Investig 1999, 103: 1489–1498. 10.1172/JCI6223
Article
CAS
Google Scholar
Strable MS, Ntambi JM: Genetic control of de novo lipogenesis: role in diet-induced obesity. Crit Rev Biochem Mol Biol 2010, 45: 199–214. 10.3109/10409231003667500
Article
CAS
Google Scholar
Jiang T, Wang Z, Proctor G, Moskowitz S, Liebman SE, Rogers T, Lucia MS, Li J, Levi M: Diet-induced obesity in C57BL/6J mice causes increased renal lipid accumulation and glomerulosclerosis via a sterol regulatory element-binding protein-1c-dependent pathway. J Biol Chem 2005, 280: 32317–32325. 10.1074/jbc.M500801200
Article
CAS
Google Scholar
Peacock WFt, Maisel A, Kim J, Ronco C: Neutrophil gelatinase associated lipocalin in acute kidney injury. Postgrad Med 2013, 125: 82–93. 10.3810/pgm.2013.11.2715
Google Scholar
Cairns AP, O'Donoghue PM, Patterson VH, Brown JH: Very-long-chain acyl-coenzyme A dehydrogenase deficiency—a new cause of myoglobinuric acute renal failure. Nephrol Dial Transplant 2000, 15: 1232–1234. 10.1093/ndt/15.8.1232
Article
CAS
Google Scholar
Krebs HA: Renal gluconeogenesis. Adv Enzyme Regul 1963, 1: 385–400. 10.1016/0065-2571(63)90034-7
Article
CAS
Google Scholar
Meyer C, Stumvoll M, Dostou J, Welle S, Haymond M, Gerich J: Renal substrate exchange and gluconeogenesis in normal postabsorptive humans. Am J Physiol Endocrinol Metab 2002, 282: E428–434.
Article
CAS
Google Scholar
Ekberg K, Landau BR, Wajngot A, Chandramouli V, Efendic S, Brunengraber H, Wahren J: Contributions by kidney and liver to glucose production in the postabsorptive state and after 60 h of fasting. Diabetes 1999, 48: 292–298. 10.2337/diabetes.48.2.292
Article
CAS
Google Scholar