Authors:
Volchenboum, SL
Mohsen, AWA
Kim, JJP
Vockley, J
Citation: Sl. Volchenboum et al., Arginine 387 of human isovaleryl-CoA dehydrogenase plays a crucial role insubstrate/product binding, MOL GEN MET, 74(1-2), 2001, pp. 226-237
Citation: Awa. Mohsen et al., Identification of Caenorhabditis elegans isovaleryl-CoA dehydrogenase and structural comparison with other acyl-CoA dehydrogenases, MOL GEN MET, 73(2), 2001, pp. 126-137
Authors:
Cox, KB
Hamm, DA
Millington, DS
Matern, D
Vockley, J
Rinaldo, P
Pinkert, CA
Rhead, WJ
Lindsey, JR
Wood, PA
Citation: Kb. Cox et al., Gestational, pathologic and biochemical differences between very long-chain acyl-CoA dehydrogenase deficiency and long-chain acyl-CoA dehydrogenase deficiency in the mouse, HUM MOL GEN, 10(19), 2001, pp. 2069-2077
Authors:
Willard, JM
Reinard, T
Mohsen, AW
Vockley, J
Citation: Jm. Willard et al., Cloning of genomic and cDNA for mouse isovaleryl-CoA dehydrogenase (IVD) and evolutionary comparison to other known IVDs, GENE, 270(1-2), 2001, pp. 253-257
Authors:
Corydon, MJ
Vockley, J
Rinaldo, P
Rhead, WJ
Kjeldsen, M
Winter, V
Riggs, C
Babovic-Vuksanovic, D
Smeitink, J
De Jong, J
Levy, H
Sewell, AC
Roe, C
Matern, D
Dasouki, M
Gregersen, N
Citation: Mj. Corydon et al., Role of common gene variations in the molecular pathogenesis of short-chain acyl-CoA dehydrogenase deficiency, PEDIAT RES, 49(1), 2001, pp. 18-23
Authors:
Matern, D
Hart, P
Murtha, AP
Vockley, J
Gregersen, N
Millington, DS
Treem, WR
Citation: D. Matern et al., Acute fatty liver of pregnancy associated with short-chain acyl-coenzyme Adehydrogenase deficiency, J PEDIAT, 138(4), 2001, pp. 585-588
Authors:
Hoard, HM
Benson, LM
Vockley, J
Naylor, S
Citation: Hm. Hoard et al., Microelectrospray ionization analysis of noncovalent interactions within the electron transferring flavoprotein, BIOC BIOP R, 282(1), 2001, pp. 297-305
Authors:
Binzak, BA
Wevers, RA
Moolenaar, SH
Lee, YM
Hwu, WL
Poggi-Bach, J
Engelki, UFH
Hoard, HM
Vockley, JG
Vockley, J
Citation: Ba. Binzak et al., Cloning of dimethylglycine dehydrogenase and a new human inborn error of metabolism, dimethylglycine dehydrogenase deficiency, AM J HU GEN, 68(4), 2001, pp. 839-847
Authors:
Le, WP
Abbas, AS
Sprecher, H
Vockley, J
Schulz, H
Citation: Wp. Le et al., Long-chain acyl-CoA dehydrogenase is a key enzyme in the mitochondrial beta-oxidation of unsaturated fatty acids, BBA-MOL C B, 1485(2-3), 2000, pp. 121-128
Authors:
Vockley, J
Rinaldo, P
Bennett, MJ
Matern, D
Vladutiu, GD
Citation: J. Vockley et al., Synergistic heterozygosity: Disease resulting from multiple partial defects in one or more metabolic pathways, MOL GEN MET, 71(1-2), 2000, pp. 10-18
Authors:
Vockley, J
Mohsen, AWA
Binzak, B
Willard, J
Fauq, A
Citation: J. Vockley et al., Mammalian branched-chain acyl-CoA dehydrogenases: Molecular cloning and characterization of recombinant enzymes, METH ENZYM, 324, 2000, pp. 241-258
Authors:
Gibson, KM
Burlingame, TG
Hogema, B
Jakobs, C
Schutgens, RBH
Millington, D
Roe, CR
Roe, DS
Sweetman, L
Steiner, RD
Linck, L
Pohowalla, P
Sacks, M
Kiss, D
Rinaldo, P
Vockley, J
Citation: Km. Gibson et al., 2-Methylbutyryl-coenzyme A dehydrogenase deficiency: A new inborn error ofL-isoleucine metabolism, PEDIAT RES, 47(6), 2000, pp. 830-833
Authors:
Treacy, EP
Lambert, DM
Barnes, R
Boriack, RL
Vockley, J
O'Brien, LK
Jones, PM
Bennett, MJ
Citation: Ep. Treacy et al., Short-chain hydroxyacyl-coenzyme A dehydrogenase deficiency presenting as unexpected infant death: A family study, J PEDIAT, 137(2), 2000, pp. 257-259
Authors:
Reinard, T
Janke, V
Willard, J
Buck, F
Jacobsen, HJ
Vockley, J
Citation: T. Reinard et al., Cloning of a gene for an acyl-CoA dehydrogenase from Pisum sativum L. and purification and characterization of its product as an isovaleryl-CoA dehydrogenase, J BIOL CHEM, 275(43), 2000, pp. 33738-33743
Citation: Sl. Volchenboum et J. Vockley, Mitochondrial import and processing of wild type and type III mutant isovaleryl-CoA dehydrogenase, J BIOL CHEM, 275(11), 2000, pp. 7958-7963
Authors:
Vockley, J
Rogan, PK
Anderson, BD
Willard, J
Seelan, RS
Smith, DI
Liu, WG
Citation: J. Vockley et al., Exon skipping in IVD RNA processing in isovaleric acidemia caused by pointmutations in the coding region of the IVD gene, AM J HU GEN, 66(2), 2000, pp. 356-367
Authors:
Tein, I
Haslam, RHA
Rhead, WJ
Bennett, MJ
Becker, LE
Vockley, J
Citation: I. Tein et al., Short-chain acyl-CoA dehydrogenase deficiency - A cause of ophthalmoplegiaand multicore myopathy, NEUROLOGY, 52(2), 1999, pp. 366-372
Authors:
Babovic-Vuksanovic, D
Patterson, MC
Schwenk, WF
O'Brien, JF
Vockley, J
Freeze, HH
Mehta, DP
Michels, VV
Citation: D. Babovic-vuksanovic et al., Severe hypoglycemia as a presenting symptom of carbohydrate-deficient glycoprotein syndrome, J PEDIAT, 135(6), 1999, pp. 775-781
Authors:
Moolenaar, SH
Poggi-Bach, J
Engelke, UFH
Corstiaensen, JMB
Heerschap, A
de Jong, JGN
Binzak, BA
Vockley, J
Wevers, RA
Citation: Sh. Moolenaar et al., Defect in dimethylglycine dehydrogenase, a new inborn error of metabolism:NMR spectroscopy study, CLIN CHEM, 45(4), 1999, pp. 459-464
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:
Kurtz, DM
Rinaldo, P
Rhead, WJ
Tian, LQ
Millington, DS
Vockley, J
Hamm, DA
Brix, AE
Lindsey, JR
Pinkert, CA
O'Brien, WE
Wood, PA
Citation: Dm. Kurtz et al., Targeted disruption of mouse long-chain acyl-CoA dehydrogenase gene reveals crucial roles for fatty acid oxidation, P NAS US, 95(26), 1998, pp. 15592-15597