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Results:
1-22
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Results: 22
Authors:
Matern, D
Shehata, BM
Shekhawat, P
Strauss, AW
Bennett, MJ
Rinaldo, P
Citation: D. Matern et al., Placental floor infarction complicating the pregnancy of a fetus with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, MOL GEN MET, 72(3), 2001, pp. 265-268
Authors:
Yoon, HR
Strauss, AW
Yoo, HW
Citation: Hr. Yoon et al., Sudden death in a Korean infant with very long-chain acyl-CoA dehydrogenase deficiency, J INH MET D, 24(3), 2001, pp. 407-408
Authors:
Albers, S
Levy, HL
Irons, M
Strauss, AW
Marsden, D
Citation: S. Albers et al., Compound heterozygosity in four asymptomatic siblings with medium-chain acyl-CoA dehydrogenase deficiency, J INH MET D, 24(3), 2001, pp. 417-418
Authors:
Wood, JC
Magera, MJ
Rinaldo, P
Seashore, MR
Strauss, AW
Friedman, A
Citation: Jc. Wood et al., Diagnosis of very long chain acyl-dehydrogenase deficiency from an infant's newborn screening card, PEDIATRICS, 108(1), 2001, pp. NIL_110-NIL_112
Authors:
Ibdah, JA
Zhao, YW
Viola, J
Gibson, B
Bennett, MJ
Strauss, AW
Citation: Ja. Ibdah et al., Molecular prenatal diagnosis in families with fetal mitochondrial trifunctional protein mutations, J PEDIAT, 138(3), 2001, pp. 396-399
Authors:
Ibdah, JA
Paul, H
Zhao, Y
Binford, S
Salleng, K
Cline, M
Matern, D
Bennett, MJ
Rinaldo, P
Strauss, AW
Citation: Ja. Ibdah et al., Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death, J CLIN INV, 107(11), 2001, pp. 1403-1409
Authors:
Barycki, JJ
O'Brien, LK
Strauss, AW
Banaszak, LJ
Citation: Jj. Barycki et al., Glutamate 170 of human L-3-hydroxyacyl-CoA dehydrogenase is required for proper orientation of the catalytic histidine and structural integrity of the enzyme, J BIOL CHEM, 276(39), 2001, pp. 36718-36726
Authors:
Jones, PM
Moffitt, M
Joseph, D
Harthcock, PA
Boriack, RL
Ibdah, JA
Strauss, AW
Bennett, MJ
Citation: Pm. Jones et al., Accumulation of free 3-hydroxy fatty acids in the culture media of fibroblasts from patients deficient in long-chain L-3-hydroxyacyl-CoA dehydrogenase: A useful diagnostic aid, CLIN CHEM, 47(7), 2001, pp. 1190-1194
Authors:
Zytkovicz, TH
Fitzgerald, EF
Marsden, D
Larson, CA
Shih, VE
Johnson, DM
Strauss, AW
Comeau, AM
Eaton, RB
Grady, GF
Citation: Th. Zytkovicz et al., Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: A two-year summary from the New Englandnewborn screening program, CLIN CHEM, 47(11), 2001, pp. 1945-1955
Authors:
Schlattner, U
Eder, M
Dolder, M
Khuchua, ZA
Strauss, AW
Wallimann, T
Citation: U. Schlattner et al., Divergent enzyme kinetics and structural properties of the two human mitochondrial creatine kinase isoenzymes, BIOL CHEM, 381(11), 2000, pp. 1063-1070
Authors:
Bennett, MJ
Rinaldo, P
Strauss, AW
Citation: Mj. Bennett et al., Inborn errors of mitochondrial fatty acid oxidation, CR R CL LAB, 37(1), 2000, pp. 1-44
Authors:
Barycki, JJ
O'Brien, LK
Strauss, AW
Banaszak, LJ
Citation: Jj. Barycki et al., Sequestration of the active site by interdomain shifting - Crystallographic and spectroscopic evidence for distinct conformations of L-3-hydroxyacyl-CoA dehydrogenase, J BIOL CHEM, 275(35), 2000, pp. 27186-27196
Authors:
Zhou, YQ
Kelly, DP
Strauss, AW
Sims, H
Zhang, ZF
Citation: Yq. Zhou et al., Characterization of the human very-long-chain acyl-CoA dehydrogenase gene promoter region: A role for activator protein 2, MOL GEN MET, 68(4), 1999, pp. 481-487
Authors:
Barycki, JJ
O'Brien, LK
Birktoft, JJ
Strauss, AW
Banaszak, LJ
Citation: Jj. Barycki et al., Pig heart short chain L-3-hydroxyacyl-CoA dehydrogenase revisited: Sequence analysis and crystal structure determination, PROTEIN SCI, 8(10), 1999, pp. 2010-2018
Authors:
Strauss, AW
Bennett, MJ
Rinaldo, P
Sims, HF
O'Brien, LK
Zhao, YW
Gibson, B
Ibdah, J
Citation: Aw. Strauss et al., Inherited long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and a fetal-maternal interaction cause maternal liver disease and other pregnancy complications, SEM PERINAT, 23(2), 1999, pp. 100-112
Authors:
Ibdah, JA
Dasouki, MJ
Strauss, AW
Citation: Ja. Ibdah et al., Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: Variable expressivity of maternal illness during pregnancy and unusual presentation with infantile cholestasis and hypocalcaemia, J INH MET D, 22(7), 1999, pp. 811-814
Authors:
Matern, D
Strauss, AW
Hillman, SL
Mayatepek, E
Millington, DS
Trefz, FK
Citation: D. Matern et al., Diagnosis of mitochondrial trifunctional protein deficiency in a blood spot from the newborn screening card by tandem mass spectrometry and DNA analysis, PEDIAT RES, 46(1), 1999, pp. 45-49
Authors:
Ibdah, JA
Bennett, MJ
Rinaldo, P
Zhao, YW
Gibson, B
Sims, HF
Strauss, AW
Citation: Ja. Ibdah et al., A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women, N ENG J MED, 340(22), 1999, pp. 1723-1731
Authors:
Hahn, SH
Lee, EH
Jung, JW
Hong, CH
Yoon, HR
Rinaldo, P
Sims, H
Gibson, B
Strauss, AW
Citation: Sh. Hahn et al., Very long chain acyl coenzyme A dehydrogenase deficiency in a 5-month-old Korean boy: Identification of a novel mutation, J PEDIAT, 135(2), 1999, pp. 250-253
Authors:
Qin, WN
Khuchua, Z
Boero, J
Payne, RM
Strauss, AW
Citation: Wn. Qin et al., Oxidative myocytes of heart and skeletal muscle express abundant sarcomeric mitochondrial creatine kinase, HISTOCHEM J, 31(6), 1999, pp. 357-365
Authors:
Mathur, A
Sims, HF
Gopalakrishnan, D
Gibson, B
Rinaldo, P
Vockley, J
Hug, G
Strauss, AW
Citation: A. Mathur et al., Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death, CIRCULATION, 99(10), 1999, pp. 1337-1343
Authors:
Barycki, JJ
O'Brien, LK
Bratt, JM
Zhang, RG
Sanishvili, R
Strauss, AW
Banaszak, LJ
Citation: Jj. Barycki et al., Biochemical characterization and crystal structure determination of human heart short chain L-3-Hydroxyacyl-CoA dehydrogenase provide insights into catalytic mechanism, BIOCHEM, 38(18), 1999, pp. 5786-5798
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