Colorado PROFILES, The Colorado Clinical and Translational Sciences Institute (CCTSI)
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Stephen I Goodman

TitleDistinguished Professor
InstitutionUniversity of Colorado Denver - Anschutz Medical Campus
DepartmentSOM-PEDS
Address2RC1N
Research North Tower
12800 E 19th Ave, Bldg. RC1 North Room P18-9103
Aurora CO 80010

     Bibliographic 
     selected publications
    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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    1. Busanello EN, Fernandes CG, Martell RV, Lobato VG, Goodman S, Woontner M, de Souza DO, Wajner M. Disturbance of the glutamatergic system by glutaric acid in striatum and cerebral cortex of glutaryl-CoA dehydrogenase-deficient knockout mice: Possible implications for the neuropathology of glutaric acidemia type I. J Neurol Sci. 2014 Nov 15; 346(1-2):260-7.
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    2. Seminotti B, Ribeiro RT, Amaral AU, da Rosa MS, Pereira CC, Leipnitz G, Koeller DM, Goodman S, Woontner M, Wajner M. Acute lysine overload provokes protein oxidative damage and reduction of antioxidant defenses in the brain of infant glutaryl-CoA dehydrogenase deficient mice: a role for oxidative stress in GA I neuropathology. J Neurol Sci. 2014 Sep 15; 344(1-2):105-13.
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    3. Lagranha VL, Matte U, de Carvalho TG, Seminotti B, Pereira CC, Koeller DM, Woontner M, Goodman SI, de Souza DO, Wajner M. Increased glutamate receptor and transporter expression in the cerebral cortex and striatum of gcdh-/- mice: possible implications for the neuropathology of glutaric acidemia type I. PLoS One. 2014; 9(3):e90477.
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    4. Busanello EN, Pettenuzzo L, Botton PH, Pandolfo P, de Souza DO, Woontner M, Goodman S, Koeller D, Wajner M. Neurodevelopmental and cognitive behavior of glutaryl-CoA dehydrogenase deficient knockout mice. Life Sci. 2013 Feb 7; 92(2):137-42.
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    5. Seminotti B, Amaral AU, da Rosa MS, Fernandes CG, Leipnitz G, Olivera-Bravo S, Barbeito L, Ribeiro CA, de Souza DO, Woontner M, Goodman SI, Koeller DM, Wajner M. Disruption of brain redox homeostasis in glutaryl-CoA dehydrogenase deficient mice treated with high dietary lysine supplementation. Mol Genet Metab. 2013 Jan; 108(1):30-9.
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    6. Amaral AU, Seminotti B, Cecatto C, Fernandes CG, Busanello EN, Zanatta Â, Kist LW, Bogo MR, de Souza DO, Woontner M, Goodman S, Koeller DM, Wajner M. Reduction of Na+, K+-ATPase activity and expression in cerebral cortex of glutaryl-CoA dehydrogenase deficient mice: a possible mechanism for brain injury in glutaric aciduria type I. Mol Genet Metab. 2012 Nov; 107(3):375-82.
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    7. Amaral AU, Cecatto C, Seminotti B, Zanatta Â, Fernandes CG, Busanello EN, Braga LM, Ribeiro CA, de Souza DO, Woontner M, Koeller DM, Goodman S, Wajner M. Marked reduction of Na(+), K(+)-ATPase and creatine kinase activities induced by acute lysine administration in glutaryl-CoA dehydrogenase deficient mice. Mol Genet Metab. 2012 Sep; 107(1-2):81-6.
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    8. Seminotti B, da Rosa MS, Fernandes CG, Amaral AU, Braga LM, Leipnitz G, de Souza DO, Woontner M, Koeller DM, Goodman S, Wajner M. Induction of oxidative stress in brain of glutaryl-CoA dehydrogenase deficient mice by acute lysine administration. Mol Genet Metab. 2012 May; 106(1):31-8.
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    9. Kölker S, Christensen E, Leonard JV, Greenberg CR, Boneh A, Burlina AB, Burlina AP, Dixon M, Duran M, García Cazorla A, Goodman SI, Koeller DM, Kyllerman M, Mühlhausen C, Müller E, Okun JG, Wilcken B, Hoffmann GF, Burgard P. Diagnosis and management of glutaric aciduria type I--revised recommendations. J Inherit Metab Dis. 2011 Jun; 34(3):677-94.
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    10. Wajner M, Goodman SI. Disruption of mitochondrial homeostasis in organic acidurias: insights from human and animal studies. J Bioenerg Biomembr. 2011 Feb; 43(1):31-8.
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    11. Al-Dirbashi OY, Kölker S, Ng D, Fisher L, Rupar T, Lepage N, Rashed MS, Santa T, Goodman SI, Geraghty MT, Zschocke J, Christensen E, Hoffmann GF, Chakraborty P. Diagnosis of glutaric aciduria type 1 by measuring 3-hydroxyglutaric acid in dried urine spots by liquid chromatography tandem mass spectrometry. J Inherit Metab Dis. 2011 Feb; 34(1):173-80.
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    12. Crombez EA, Cederbaum SD, Spector E, Chan E, Salazar D, Neidich J, Goodman S. Maternal glutaric acidemia, type I identified by newborn screening. Mol Genet Metab. 2008 May; 94(1):132-4.
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    13. Keyser B, Glatzel M, Stellmer F, Kortmann B, Lukacs Z, Kölker S, Sauer SW, Muschol N, Herdering W, Thiem J, Goodman SI, Koeller DM, Ullrich K, Braulke T, Mühlhausen C. Transport and distribution of 3-hydroxyglutaric acid before and during induced encephalopathic crises in a mouse model of glutaric aciduria type 1. Biochim Biophys Acta. 2008 Jun; 1782(6):385-90.
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    14. Arnold GL, Koeberl DD, Matern D, Barshop B, Braverman N, Burton B, Cederbaum S, Fiegenbaum A, Garganta C, Gibson J, Goodman SI, Harding C, Kahler S, Kronn D, Longo N. A Delphi-based consensus clinical practice protocol for the diagnosis and management of 3-methylcrotonyl CoA carboxylase deficiency. Mol Genet Metab. 2008 Apr; 93(4):363-70.
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    15. Zinnanti WJ, Lazovic J, Housman C, LaNoue K, O'Callaghan JP, Simpson I, Woontner M, Goodman SI, Connor JR, Jacobs RE, Cheng KC. Mechanism of age-dependent susceptibility and novel treatment strategy in glutaric acidemia type I. J Clin Invest. 2007 Nov; 117(11):3258-70.
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    16. Stellmer F, Keyser B, Burckhardt BC, Koepsell H, Streichert T, Glatzel M, Jabs S, Thiem J, Herdering W, Koeller DM, Goodman SI, Lukacs Z, Ullrich K, Burckhardt G, Braulke T, Mühlhausen C. 3-Hydroxyglutaric acid is transported via the sodium-dependent dicarboxylate transporter NaDC3. J Mol Med (Berl). 2007 Jul; 85(7):763-70.
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    17. Kölker S, Christensen E, Leonard JV, Greenberg CR, Burlina AB, Burlina AP, Dixon M, Duran M, Goodman SI, Koeller DM, Müller E, Naughten ER, Neumaier-Probst E, Okun JG, Kyllerman M, Surtees RA, Wilcken B, Hoffmann GF, Burgard P. Guideline for the diagnosis and management of glutaryl-CoA dehydrogenase deficiency (glutaric aciduria type I). J Inherit Metab Dis. 2007 Feb; 30(1):5-22.
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    18. Woontner M, Goodman SI. Chromatographic analysis of amino and organic acids in physiological fluids to detect inborn errors of metabolism. Curr Protoc Hum Genet. 2006 Nov; Chapter 17:Unit 17.2.
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    19. Bjugstad KB, Crnic LS, Goodman SI, Freed CR. Infant mice with glutaric acidaemia type I have increased vulnerability to 3-nitropropionic acid toxicity. J Inherit Metab Dis. 2006 Oct; 29(5):612-9.
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    20. Zinnanti WJ, Lazovic J, Wolpert EB, Antonetti DA, Smith MB, Connor JR, Woontner M, Goodman SI, Cheng KC. New insights for glutaric aciduria type I. Brain. 2006 Aug; 129(Pt 8):e55.
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    21. Deignan JL, Livesay JC, Yoo PK, Goodman SI, O'Brien WE, Iyer RK, Cederbaum SD, Grody WW. Ornithine deficiency in the arginase double knockout mouse. Mol Genet Metab. 2006 Sep-Oct; 89(1-2):87-96.
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    22. Sauer SW, Okun JG, Fricker G, Mahringer A, Müller I, Crnic LR, Mühlhausen C, Hoffmann GF, Hörster F, Goodman SI, Harding CO, Koeller DM, Kölker S. Intracerebral accumulation of glutaric and 3-hydroxyglutaric acids secondary to limited flux across the blood-brain barrier constitute a biochemical risk factor for neurodegeneration in glutaryl-CoA dehydrogenase deficiency. J Neurochem. 2006 May; 97(3):899-910.
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    23. Magera MJ, Gunawardena ND, Hahn SH, Tortorelli S, Mitchell GA, Goodman SI, Rinaldo P, Matern D. Quantitative determination of succinylacetone in dried blood spots for newborn screening of tyrosinemia type I. Mol Genet Metab. 2006 May; 88(1):16-21.
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    24. Zinnanti WJ, Lazovic J, Wolpert EB, Antonetti DA, Smith MB, Connor JR, Woontner M, Goodman SI, Cheng KC. A diet-induced mouse model for glutaric aciduria type I. Brain. 2006 Apr; 129(Pt 4):899-910.
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    25. Gallagher RC, Cowan TM, Goodman SI, Enns GM. Glutaryl-CoA dehydrogenase deficiency and newborn screening: retrospective analysis of a low excretor provides further evidence that some cases may be missed. Mol Genet Metab. 2005 Nov; 86(3):417-20.
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    26. Sauer SW, Okun JG, Schwab MA, Crnic LR, Hoffmann GF, Goodman SI, Koeller DM, Kölker S. Bioenergetics in glutaryl-coenzyme A dehydrogenase deficiency: a role for glutaryl-coenzyme A. J Biol Chem. 2005 Jun 10; 280(23):21830-6.
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    27. Goodman SI. Development of pathogenic concepts in glutaryl-CoA dehydrogenase deficiency: the challenge. J Inherit Metab Dis. 2004; 27(6):801-3.
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    28. Kölker S, Strauss KA, Goodman SI, Hoffmann GF, Okun JG, Koeller DM. Challenges for basic research in glutaryl-CoA dehydrogenase deficiency. J Inherit Metab Dis. 2004; 27(6):843-9.
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    29. Mühlhausen C, Ergün S, Strauss KA, Koeller DM, Crnic L, Woontner M, Goodman SI, Ullrich K, Braulke T. Vascular dysfunction as an additional pathomechanism in glutaric aciduria type I. J Inherit Metab Dis. 2004; 27(6):829-34.
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    30. Naughten ER, Mayne PD, Monavari AA, Goodman SI, Sulaiman G, Croke DT. Glutaric aciduria type I: outcome in the Republic of Ireland. J Inherit Metab Dis. 2004; 27(6):917-20.
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    31. Koeller DM, Sauer S, Wajner M, de Mello CF, Goodman SI, Woontner M, Mühlhausen C, Okun JG, Kölker S. Animal models for glutaryl-CoA dehydrogenase deficiency. J Inherit Metab Dis. 2004; 27(6):813-8.
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    32. Surendran S, Bamforth FJ, Chan A, Tyring SK, Goodman SI, Matalon R. Mild elevation of N-acetylaspartic acid and macrocephaly: diagnostic problem. J Child Neurol. 2003 Nov; 18(11):809-12.
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    33. Westover JB, Goodman SI, Frerman FE. Pathogenic mutations in the carboxyl-terminal domain of glutaryl-CoA dehydrogenase: effects on catalytic activity and the stability of the tetramer. Mol Genet Metab. 2003 Aug; 79(4):245-56.
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    34. Goodman SI, Binard RJ, Woontner MR, Frerman FE. Glutaric acidemia type II: gene structure and mutations of the electron transfer flavoprotein:ubiquinone oxidoreductase (ETF:QO) gene. Mol Genet Metab. 2002 Sep-Oct; 77(1-2):86-90.
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    35. Koeller DM, Woontner M, Crnic LS, Kleinschmidt-DeMasters B, Stephens J, Hunt EL, Goodman SI. Biochemical, pathologic and behavioral analysis of a mouse model of glutaric acidemia type I. Hum Mol Genet. 2002 Feb 15; 11(4):347-57.
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    36. Wajne M, Vargas CR, Funayama C, Fernandez A, Elias ML, Goodman SI, Jakobs C, van der Knaap MS. D-2-Hydroxyglutaric aciduria in a patient with a severe clinical phenotype and unusual MRI findings. J Inherit Metab Dis. 2002 Feb; 25(1):28-34.
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    37. Rao KS, Vander Velde D, Dwyer TM, Goodman SI, Frerman FE. Alternate substrates of human glutaryl-CoA dehydrogenase: structure and reactivity of substrates, and identification of a novel 2-enoyl-CoA product. Biochemistry. 2002 Jan 29; 41(4):1274-84.
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    38. Jones PM, Tjoa S, Fennessey PV, Goodman SI, Bennett MJ. Addition of quantitative 3-hydroxy-octadecanoic acid to the stable isotope gas chromatography-mass spectrometry method for measuring 3-hydroxy fatty acids. Clin Chem. 2002 Jan; 48(1):176-9.
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    39. Goodman SI. Prenatal diagnosis of glutaric acidemias. Prenat Diagn. 2001 Dec; 21(13):1167-8.
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    40. Ojwang PJ, Pegoraro RJ, Deppe WM, Sankar R, McKerrow N, Varughese L, Stoker AF, Goodman SI. Biochemical and molecular diagnosis of glutaric aciduria type 1 in a black South African male child: case report. East Afr Med J. 2001 Dec; 78(12):682-5.
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    41. Westover JB, Goodman SI, Frerman FE. Binding, hydration, and decarboxylation of the reaction intermediate glutaconyl-coenzyme A by human glutaryl-CoA dehydrogenase. Biochemistry. 2001 Nov 20; 40(46):14106-14.
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    42. Huang TT, Carlson EJ, Kozy HM, Mantha S, Goodman SI, Ursell PC, Epstein CJ. Genetic modification of prenatal lethality and dilated cardiomyopathy in Mn superoxide dismutase mutant mice. Free Radic Biol Med. 2001 Nov 1; 31(9):1101-10.
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    43. Bjugstad KB, Goodman SI, Freed CR. Age at symptom onset predicts severity of motor impairment and clinical outcome of glutaric acidemia type 1. J Pediatr. 2000 Nov; 137(5):681-6.
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    44. Dwyer TM, Rao KS, Goodman SI, Frerman FE. Proton abstraction reaction, steady-state kinetics, and oxidation-reduction potential of human glutaryl-CoA dehydrogenase. Biochemistry. 2000 Sep 19; 39(37):11488-99.
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    45. Jones PM, Quinn R, Fennessey PV, Tjoa S, Goodman SI, Fiore S, Burlina AB, Rinaldo P, Boriack RL, Bennett MJ. Improved stable isotope dilution-gas chromatography-mass spectrometry method for serum or plasma free 3-hydroxy-fatty acids and its utility for the study of disorders of mitochondrial fatty acid beta-oxidation. Clin Chem. 2000 Feb; 46(2):149-55.
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    46. Enns GM, Bennett MJ, Hoppel CL, Goodman SI, Weisiger K, Ohnstad C, Golabi M, Packman S. Mitochondrial respiratory chain complex I deficiency with clinical and biochemical features of long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. J Pediatr. 2000 Feb; 136(2):251-4.
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    47. Spector EB, Seltzer WK, Goodman SI. Assignment of electron transfer flavoprotein-ubiquinone oxidoreductase (ETF-QO) to human chromosome 4q33 by fluorescence in situ hybridization and somatic cell hybridization. Mol Genet Metab. 1999 Aug; 67(4):364-7.
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    48. Baric I, Zschocke J, Christensen E, Duran M, Goodman SI, Leonard JV, Müller E, Morton DH, Superti-Furga A, Hoffmann GF. Diagnosis and management of glutaric aciduria type I. J Inherit Metab Dis. 1998 Jun; 21(4):326-40.
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    49. Goodman SI, Stein DE, Schlesinger S, Christensen E, Schwartz M, Greenberg CR, Elpeleg ON. Glutaryl-CoA dehydrogenase mutations in glutaric acidemia (type I): review and report of thirty novel mutations. Hum Mutat. 1998; 12(3):141-4.
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    50. Biery BJ, Stein DE, Morton DH, Goodman SI. Gene structure and mutations of glutaryl-coenzyme A dehydrogenase: impaired association of enzyme subunits that is due to an A421V substitution causes glutaric acidemia type I in the Amish. Am J Hum Genet. 1996 Nov; 59(5):1006-11.
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    51. Goodman SI, Kratz LE, DiGiulio KA, Biery BJ, Goodman KE, Isaya G, Frerman FE. Cloning of glutaryl-CoA dehydrogenase cDNA, and expression of wild type and mutant enzymes in Escherichia coli. Hum Mol Genet. 1995 Sep; 4(9):1493-8.
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    52. Koeller DM, DiGiulio KA, Angeloni SV, Dowler LL, Frerman FE, White RA, Goodman SI. Cloning, structure, and chromosome localization of the mouse glutaryl-CoA dehydrogenase gene. Genomics. 1995 Aug 10; 28(3):508-12.
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    53. Greenberg CR, Reimer D, Singal R, Triggs-Raine B, Chudley AE, Dilling LA, Philipps S, Haworth JC, Seargeant LE, Goodman SI. A G-to-T transversion at the +5 position of intron 1 in the glutaryl CoA dehydrogenase gene is associated with the Island Lake variant of glutaric acidemia type I. Hum Mol Genet. 1995 Mar; 4(3):493-5.
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    54. Beard SE, Goodman SI, Bemelen K, Frerman FE. Characterization of a mutation that abolishes quinone reduction by electron transfer flavoprotein-ubiquinone oxidoreductase. Hum Mol Genet. 1995 Feb; 4(2):157-61.
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    55. Herrick KR, Salazar D, Goodman SI, Finocchiaro G, Bedzyk LA, Frerman FE. Expression and characterization of human and chimeric human-Paracoccus denitrificans electron transfer flavoproteins. J Biol Chem. 1994 Dec 23; 269(51):32239-45.
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    56. Goodman SI, Greene CL. Metabolic disorders of the newborn. Pediatr Rev. 1994 Sep; 15(9):359-65; quiz 365.
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    57. Greenberg CR, Duncan AM, Gregory CA, Singal R, Goodman SI. Assignment of human glutaryl-CoA dehydrogenase gene (GCDH) to the short arm of chromosome 19 (19p13.2) by in situ hybridization and somatic cell hybrid analysis. Genomics. 1994 May 1; 21(1):289-90.
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    58. Haworth JC, Singal R, Goodman SI, Greenberg CR. TaqI polymorphism in intron 2 of the GCDH gene. Hum Mol Genet. 1994 Apr; 3(4):678.
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    59. Goodman SI, Axtell KM, Bindoff LA, Beard SE, Gill RE, Frerman FE. Molecular cloning and expression of a cDNA encoding human electron transfer flavoprotein-ubiquinone oxidoreductase. Eur J Biochem. 1994 Jan 15; 219(1-2):277-86.
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    60. Arnold GL, Greene CL, Stout JP, Goodman SI. Molybdenum cofactor deficiency. J Pediatr. 1993 Oct; 123(4):595-8.
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    61. Colombo I, DiDonato S, Volta M, Gellera C, Garavaglia B, Montermini L, Yamaguchi S, Goodman SI, Frerman FE, Finocchiaro G. Molecular heterogeneity of beta-ETF deficiency in glutaric aciduria type II. Prog Clin Biol Res. 1992; 375:561-6.
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    62. Goodman SI, Kratz LE, Frerman FE. Pork and human cDNAs encoding glutaryl-CoA dehydrogenase. Prog Clin Biol Res. 1992; 375:169-73.
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    63. Blom HJ, Davidson AJ, Finkelstein JD, Luder AS, Bernardini I, Martin JJ, Tangerman A, Trijbels JM, Mudd SH, Goodman SI, et al. Persistent hypermethioninaemia with dominant inheritance. J Inherit Metab Dis. 1992; 15(2):188-97.
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    64. Goodman SI, Bemelen KF, Frerman FE. Human cDNA encoding ETF dehydrogenase (ETF:ubiquinone oxido-reductase), and mutations in glutaric acidemia type II. Prog Clin Biol Res. 1992; 375:567-72.
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    65. Kler RS, Jackson S, Bartlett K, Bindoff LA, Eaton S, Pourfarzam M, Frerman FE, Goodman SI, Watmough NJ, Turnbull DM. Quantitation of acyl-CoA and acylcarnitine esters accumulated during abnormal mitochondrial fatty acid oxidation. J Biol Chem. 1991 Dec 5; 266(34):22932-8.
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    66. Goodman SI, Greene CL. Inborn errors as causes of acute disease in infancy. Semin Perinatol. 1991 Feb; 15(1 Suppl 1):31-4.
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    67. Bennett MJ, Pollitt RJ, Goodman SI, Hale DE, Vamecq J. Atypical riboflavin-responsive glutaric aciduria, and deficient peroxisomal glutaryl-CoA oxidase activity: a new peroxisomal disorder. J Inherit Metab Dis. 1991; 14(2):165-73.
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    68. Haworth JC, Booth FA, Chudley AE, deGroot GW, Dilling LA, Goodman SI, Greenberg CR, Mallory CJ, McClarty BM, Seshia SS, et al. Phenotypic variability in glutaric aciduria type I: Report of fourteen cases in five Canadian Indian kindreds. J Pediatr. 1991 Jan; 118(1):52-8.
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    69. Bell RB, Brownell AK, Roe CR, Engel AG, Goodman SI, Frerman FE, Seccombe DW, Snyder FF. Electron transfer flavoprotein: ubiquinone oxidoreductase (ETF:QO) deficiency in an adult. Neurology. 1990 Nov; 40(11):1779-82.
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    70. Loehr JP, Goodman SI, Frerman FE. Glutaric acidemia type II: heterogeneity of clinical and biochemical phenotypes. Pediatr Res. 1990 Mar; 27(3):311-5.
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    71. Goodman SI, Frerman FE. Progress in cloning the gene for electron transfer flavoprotein:ubiquinone oxidoreductase. Prog Clin Biol Res. 1990; 321:653-7.
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    72. Goodman SI, Loehr JP, Frerman FE. Clinical and biochemical aspects of glutaric acidemia type II. Prog Clin Biol Res. 1990; 321:465-76.
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    73. Kamiya M, Eimoto T, Kishimoto H, Tsudzuki T, Morishita H, Wada Y, Wakabayashi T, Hashimoto T, Goodman SI, Frerman FE, et al. Glutaric aciduria type II: autopsy study of a case with electron-transferring flavoprotein dehydrogenase deficiency. Pediatr Pathol. 1990; 10(6):1007-19.
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    74. Luder AS, Davidson A, Goodman SI, Greene CL. Transient nonketotic hyperglycinemia in neonates. J Pediatr. 1989 Jun; 114(6):1013-5.
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    75. Gibson KM, Goodman SI, Frerman FE, Glasgow AM. Succinic semialdehyde dehydrogenase deficiency associated with combined 4-hydroxybutyric and dicarboxylic acidurias: potential for clinical misdiagnosis based on urinary organic acid profiling. J Pediatr. 1989 Apr; 114(4 Pt 1):607-10.
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    76. Wilson GN, de Chadarévian JP, Kaplan P, Loehr JP, Frerman FE, Goodman SI. Glutaric aciduria type II: review of the phenotype and report of an unusual glomerulopathy. Am J Med Genet. 1989 Mar; 32(3):395-401.
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    77. Bergman I, Finegold D, Gartner JC, Zitelli BJ, Claassen D, Scarano J, Roe CR, Stanley C, Goodman SI. Acute profound dystonia in infants with glutaric acidemia. Pediatrics. 1989 Feb; 83(2):228-34.
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    78. Chow CW, Frerman FE, Goodman SI, Brown GK, Pitt JJ, Danks DM. Striatal degeneration in glutaric acidaemia type II. Acta Neuropathol. 1989; 77(5):554-6.
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    79. Perelmuter B, Goodman SI, McCabe ER. Galactosaemia with fatal cerebral oedema. J Inherit Metab Dis. 1989; 12(4):489-90.
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    80. Rinaldo P, O'Shea JJ, Goodman SI, Miller LV, Fennessey PV, Whelan DT, Hill RE, Tanaka K. Comparison of urinary acylglycines and acylcarnitines as diagnostic markers of medium-chain acyl-CoA dehydrogenase deficiency. J Inherit Metab Dis. 1989; 12 Suppl 2:325-8.
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    81. Treem WR, Stanley CA, Goodman SI. Medium-chain acyl-CoA dehydrogenase deficiency: metabolic effects and therapeutic efficacy of long-term L-carnitine supplementation. J Inherit Metab Dis. 1989; 12(2):112-9.
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    82. Gibson KM, Hoffmann G, Nyhan WL, Aramaki S, Thompson JA, Goodman SI, Johnson DA, Fife RR. 4-Hydroxybutyric aciduria in a patient without ataxia or convulsions. Eur J Pediatr. 1988 Jun; 147(5):529-31.
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    83. Chow CW, Haan EA, Goodman SI, Anderson RM, Evans WA, Kleinschmidt-DeMasters BK, Wise G, McGill JJ, Danks DM. Neuropathology in glutaric acidaemia type 1. Acta Neuropathol. 1988; 76(6):590-4.
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    84. Lipkin PH, Roe CR, Goodman SI, Batshaw ML. A case of glutaric acidemia type I: effect of riboflavin and carnitine. J Pediatr. 1988 Jan; 112(1):62-5.
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    85. Goodman SI, Frerman FE, Loehr JP. Recent progress in understanding glutaric acidemias. Enzyme. 1987; 38(1-4):76-9.
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    86. Lenich AC, Goodman SI. The purification and characterization of glutaryl-coenzyme A dehydrogenase from porcine and human liver. J Biol Chem. 1986 Mar 25; 261(9):4090-6.
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    87. Hale DE, Batshaw ML, Coates PM, Frerman FE, Goodman SI, Singh I, Stanley CA. Long-chain acyl coenzyme A dehydrogenase deficiency: an inherited cause of nonketotic hypoglycemia. Pediatr Res. 1985 Jul; 19(7):666-71.
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    88. Frerman FE, Goodman SI. Deficiency of electron transfer flavoprotein or electron transfer flavoprotein:ubiquinone oxidoreductase in glutaric acidemia type II fibroblasts. Proc Natl Acad Sci U S A. 1985 Jul; 82(13):4517-20.
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    89. Frerman FE, Goodman SI. Fluorometric assay of acyl-CoA dehydrogenases in normal and mutant human fibroblasts. Biochem Med. 1985 Feb; 33(1):38-44.
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    90. Matalon R, Stumpf DA, Michals K, Hart RD, Parks JK, Goodman SI. Lipoamide dehydrogenase deficiency with primary lactic acidosis: favorable response to treatment with oral lipoic acid. J Pediatr. 1984 Jan; 104(1):65-9.
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    91. Goodman SI, Frerman FE. Glutaric acidaemia type II (multiple acyl-CoA dehydrogenation deficiency). J Inherit Metab Dis. 1984; 7 Suppl 1:33-7.
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    92. Goodman SI, Norenberg MD. Glutaric acidemia as a cause of striatal necrosis in childhood. Ann Neurol. 1983 May; 13(5):582-3.
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    93. Goodman SI, Reale M, Berlow S. Glutaric acidemia type II: a form with deleterious intrauterine effects. J Pediatr. 1983 Mar; 102(3):411-3.
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    94. McCabe ER, Goodman SI, Fennessey PV, Miles BS, Wall M, Silverman A. Glutaric, 3-hydroxypropionic, and lactic aciduria with metabolic acidemia, following extensive small bowel resection. Biochem Med. 1982 Oct; 28(2):229-36.
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    95. Goodman SI, Stene DO, McCabe ER, Norenberg MD, Shikes RH, Stumpf DA, Blackburn GK. Glutaric acidemia type II: clinical, biochemical, and morphologic considerations. J Pediatr. 1982 Jun; 100(6):946-50.
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    96. Goodman SI. Organic aciduria in the riboflavin-deficient rat. Am J Clin Nutr. 1981 Nov; 34(11):2434-7.
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    97. Goodman SI, Markey SP. Diagnosis of organic acidemias by gas chromatography--mass spectrometry. Lab Res Methods Biol Med. 1981; 6:1-158.
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    98. Leibel RL, Shih VE, Goodman SI, Bauman ML, McCabe ER, Zwerdling RG, Bergman I, Costello C. Glutaric acidemia: a metabolic disorder causing progressive choreoathetosis. Neurology. 1980 Nov; 30(11):1163-8.
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    99. Goodman SI. An introduction to gas chromatography-mass spectrometry and the inherited organic acidemias. Am J Hum Genet. 1980 Nov; 32(6):781-92.
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    100. Gabow PA, Kaehny WD, Fennessey PV, Goodman SI, Gross PA, Schrier RW. Diagnostic importance of an increased serum anion gap. N Engl J Med. 1980 Oct 9; 303(15):854-8.
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    101. Goodman SI, Gallegos DA, Pullin CJ, Halpern B, Truscott RJ, Wise G, Wilcken B, Ryan ED, Whelen DT. Antenatal diagnosis of glutaric acidemia. Am J Hum Genet. 1980 Sep; 32(5):695-9.
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    102. Goodman SI, McCabe ER, Fennessey PV, Mace JW. Multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type II) with transient hypersarcosinemia and sarcosinuria; possible inherited deficiency of an electron transfer flavoprotein. Pediatr Res. 1980 Jan; 14(1):12-7.
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    103. Valle D, Goodman SI, Harris SC, Phang JM. Genetic evidence for a common enzyme catalyzing the second step in the degradation of proline and hydroxyproline. J Clin Invest. 1979 Nov; 64(5):1365-70.
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    104. Goodman SI, McCabe ER, Fennessey PV, Miles BS, Mace JW, Jellum E. Methylmalonic/beta-hydroxy-n-valeric aciduria due to methylmalonyl-CoA mutase deficiency. Clin Chim Acta. 1978 Aug 1; 87(3):441-9.
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    105. Shafai T, Sweetman L, Weyler W, Goodman SI, Fennessey PV, Nyhan WL. Propionic acidemia with severe hyperammonemia and defective glycine metabolism. J Pediatr. 1978 Jan; 92(1):84-6.
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    106. Goodman SI, Helland P, Stokke O, Flatmark A, Jellum E. Organic acid profiles of human tissue biopsies by capillary gas chromatography-mass spectrometry. J Chromatogr. 1977 Nov 11; 142:497-503.
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    107. McCabe ER, Fennessey PV, Guggenheim MA, Miles BS, Bullen WW, Sceats DJ, Goodman SI. Human glycerol kinase deficiency with hyperglycerolemia and glyceroluria. Biochem Biophys Res Commun. 1977 Oct 24; 78(4):1327-33.
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    108. Goodman SI, Norenberg MD, Shikes RH, Breslich DJ, Moe PG. Glutaric aciduria: biochemical and morphologic considerations. J Pediatr. 1977 May; 90(5):746-50.
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    109. Valle D, Goodman SI, Applegarth DA, Shih VE, Phang JM. Type II hyperprolinemia. Delta1-pyrroline-5-carboxylic acid dehydrogenase deficiency in cultured skin fibroblasts and circulating lymphocytes. J Clin Invest. 1976 Sep; 58(3):598-603.
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    110. Stokke O, Goodman SI, Moe PG. Inhibition of brain glutamate decarboxylase by glutarate, glutaconate, and beta-hydroxyglutarate: explanation of the symptoms in glutaric aciduria? Clin Chim Acta. 1976 Feb 2; 66(3):411-5.
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    111. Bindel TH, Fennessey PV, Miles BS, Goodman SI. 4-hydroxycyclohexane-1-carboxylic acid: an unusual compound isolated from the urine of children with suspected disorders of metabolism. Clin Chim Acta. 1976 Jan 16; 66(2):209-17.
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    112. Goodman SI, Kohlhoff JG. Glutaric aciduria: inherited deficiency of glutaryl-CoA dehydrogenase activity. Biochem Med. 1975 Jun; 13(2):138-40.
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    113. Stokke O, Goodman SI, Thompson JA, Miles BS. Glutaric aciduria; presence of glutaconic and beta-hydroxyglutaric acids in urine. Biochem Med. 1975 Apr; 12(4):386-91.
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    114. Goodman SI, Markey SP, Moe PG, Miles BS, Teng CC. Glutaric aciduria; a "new" disorder of amino acid metabolism. Biochem Med. 1975 Jan; 12(1):12-21.
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    115. Goodman SI, Mace JW, Turner B, Garrett WJ. Antenatal diagnosis of argininosuccinic aciduria. Clin Genet. 1973; 4(3):236-40.
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