Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders von Djouadi, F., Aubey, F., Schlemmer, D., Ruiter, J.P.N., Wanders, R.J.A., Strauss, A.W., Bastin, Jean

Volltext

Tandem Mass Spectrometric Analysis for Amino, Organic, and Fatty Acid Disorders in Newborn Dried Blood Spots: A Two-Year Summary from the New England Newborn Screening Program von Zytkovicz, Thomas H, Fitzgerald, Eileen F, Marsden, Deborah, Larson, Cecilia A, Shih, Vivian E, Johnson, Donna M, Strauss, Arnold W, Comeau, Anne Marie, Eaton, Roger B, Grady, George F

Volltext

A Fetal Fatty-Acid Oxidation Disorder as a Cause of Liver Disease in Pregnant Women von Ibdah, Jamal A, Bennett, Michael J, Rinaldo, Piero, Zhao, Yiwen, Gibson, Beverly, Sims, Harold F, Strauss, Arnold W

Volltext

Deletion of the n-terminus of murine map2 by gene targeting disrupts hippocampal ca1 neuron architecture and alters contextual memory von Khuchua, Z., Wozniak, D.F., Bardgett, M.E., Yue, Z., McDonald, M., Boero, J., Hartman, R.E., Sims, H., Strauss, A.W.

Volltext

Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress von Spiekerkoetter, U., Tokunaga, C., Wendel, U., Mayatepek, E., Exil, V., Duran, M., Wijburg, F. A., Wanders, R. J. A., Strauss, A. W.

Volltext

Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death von MATHUR, A, SIMS, H. F, GOPALAKRISHNAN, D, GIBSON, B, RINALDO, P, VOCKLEY, J, HUG, G, STRAUSS, A. W

Volltext

Rapid, comprehensive screening of the human medium chain acyl-CoA dehydrogenase gene von McKinney, J.T, Longo, N, Hahn, S.H, Matern, D, Rinaldo, P, Strauss, A.W, Dobrowolski, S.F

Volltext

Carnitine supplementation induces long‐chain acylcarnitine production—Studies in the VLCAD‐deficient mouse von Liebig, M., Gyenes, M., Brauers, G., Ruiter, J. P. N., Wendel, U., Mayatepek, E., Strauss, A. W., Wanders, R. J. A., Spiekerkoetter, U.

Volltext

Diagnosis of Very Long Chain Acyl-Dehydrogenase Deficiency From an Infant's Newborn Screening Card von Wood, John C, Magera, Mark J, Rinaldo, Piero, Seashore, Margretta Reed, Strauss, Arnold W, Friedman, Alan

Volltext

Potential of fibrates in the treatment of fatty acid oxidation disorders: Revival of classical drugs? von Djouadi, F., Aubey, F., Schlemmer, D., Gobin, S., Laforet, P., Wanders, R. J. A., Strauss, A. W., Bonnefont, J. P., Bastin, J.

Volltext

The Molecular Basis of Pediatric Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency Associated with Maternal Acute Fatty Liver of Pregnancy von Sims, Harold F., Brackett, Jeffrey C., Powell, Cynthia K., Treem, William R., Hale, Daniel E., Bennett, Michael J., Gibson, Beverly, Shapiro, Scott, Strauss, Arnold W.

Volltext

The early‐onset phenotype of mitochondrial trifunctional protein deficiency: A lethal disorder with multiple tissue involvement von Spiekerkoetter, U., Khuchua, Z., Yue, Z., Strauss, A. W.

Volltext

Stability of the Camassa-Holm solitons von Constantin, Adrian, Strauss, WA

Volltext

Long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiency: Variable expressivity of maternal illness during pregnancy and unusual presentation with infantile cholestasis and hypocalcae... von Ibdah, J. A., Dasouki, M. J., Strauss, A. W.

Volltext

Molecular Basis of Human Mitochondrial Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency Causing Cardiomyopathy and Sudden Death in Childhood von Strauss, Arnold W., Powell, Cynthia K., Hale, Daniel E., Anderson, Melissa M., Ahuja, Ajay, Brackett, Jeffrey C., Sims, Harold F.

Volltext

Restricted neuronal expression of ubiquitous mitochondrial creatine kinase: changing patterns in development and with increased activity von Boero, J, Qin, W, Cheng, J, Woolsey, T A, Strauss, A W, Khuchua, Z

Volltext

Toward a molecular understanding of congenital heart disease von PAYNE, R. M, JOHNSON, M. C, GRANT, J. W, STRAUSS, A. W

Volltext

Reduced penetrance, variable expressivity, and genetic heterogeneity of familial atrial septal defects von BENSON, D. W, SHARKEY, A, FATKIN, D, LANG, P, BASSON, C. T, MCDONOUGH, B, STRAUSS, A. W, SEIDMAN, J. G, SEIDMAN, C. E

Volltext

Inherited long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and a fetal-maternal interaction cause maternal liver disease and other pregnancy complications von Strauss, Arnold W., Bennett, Michael J., Rinaldo, Piero, Sims, Harold F., O'Brien, Laurie K., Zhao, Yiwen, Gibson, Beverly, Ibdah, Jamal

Volltext

Cloning and characterization of human pancreatic lipase cDNA von LOWE, M. E, ROSENBLUM, J. L, STRAUSS, A. W

Volltext