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Items: 1 to 50 of 137

1.

Human hepatocyte transplantation corrects the inherited metabolic liver disorder arginase deficiency in mice.

Angarita SAK, Truong B, Khoja S, Nitzahn M, Rajbhandari AK, Zhuravka I, Duarte S, Lin MG, Lam AK, Cederbaum SD, Lipshutz GS.

Mol Genet Metab. 2018 Jun;124(2):114-123. doi: 10.1016/j.ymgme.2018.04.005. Epub 2018 Apr 21.

2.

Biopsy-proven Hepatocellular Carcinoma in a 53-year-old Woman With Arginase Deficiency.

Koo M, Lipshutz GS, Cederbaum SD, Lassman C.

Pediatr Dev Pathol. 2017 Nov-Dec;20(6):517-521. doi: 10.1177/1093526617697058. Epub 2017 Mar 7.

PMID:
29187023
3.

Biochemical characteristics of newborns with carnitine transporter defect identified by newborn screening in California.

Gallant NM, Leydiker K, Wilnai Y, Lee C, Lorey F, Feuchtbaum L, Tang H, Carter J, Enns GM, Packman S, Lin HJ, Wilcox WR, Cederbaum SD, Abdenur JE.

Mol Genet Metab. 2017 Nov;122(3):76-84. doi: 10.1016/j.ymgme.2017.06.015. Epub 2017 Jul 8.

PMID:
28711408
4.

Newborn screening for hyperargininemia due to arginase 1 deficiency.

Therrell BL, Currier R, Lapidus D, Grimm M, Cederbaum SD.

Mol Genet Metab. 2017 Aug;121(4):308-313. doi: 10.1016/j.ymgme.2017.06.003. Epub 2017 Jun 20.

PMID:
28659245
5.

Restoring Ureagenesis in Hepatocytes by CRISPR/Cas9-mediated Genomic Addition to Arginase-deficient Induced Pluripotent Stem Cells.

Lee PC, Truong B, Vega-Crespo A, Gilmore WB, Hermann K, Angarita SA, Tang JK, Chang KM, Wininger AE, Lam AK, Schoenberg BE, Cederbaum SD, Pyle AD, Byrne JA, Lipshutz GS.

Mol Ther Nucleic Acids. 2016 Nov 29;5(11):e394. doi: 10.1038/mtna.2016.98.

6.

Rescue of the Functional Alterations of Motor Cortical Circuits in Arginase Deficiency by Neonatal Gene Therapy.

Cantero G, Liu XB, Mervis RF, Lazaro MT, Cederbaum SD, Golshani P, Lipshutz GS.

J Neurosci. 2016 Jun 22;36(25):6680-90. doi: 10.1523/JNEUROSCI.0897-16.2016.

7.

Minimal ureagenesis is necessary for survival in the murine model of hyperargininemia treated by AAV-based gene therapy.

Hu C, Tai DS, Park H, Cantero G, Chan E, Yudkoff M, Cederbaum SD, Lipshutz GS.

Gene Ther. 2015 Feb;22(2):216. doi: 10.1038/gt.2014.127. No abstract available.

PMID:
25652179
8.

Minimal ureagenesis is necessary for survival in the murine model of hyperargininemia treated by AAV-based gene therapy.

Hu C, Tai DS, Park H, Cantero G, Chan E, Yudkoff M, Cederbaum SD, Lipshutz GS.

Gene Ther. 2015 Feb;22(2):111-5. doi: 10.1038/gt.2014.106. Epub 2014 Dec 4. Erratum in: Gene Ther. 2015 Feb;22(2):216. Cantero-Nieto, G [corrected to Cantero, G]. Gene Ther. 2015 Feb;22(2):216.

9.

Myocyte-mediated arginase expression controls hyperargininemia but not hyperammonemia in arginase-deficient mice.

Hu C, Kasten J, Park H, Bhargava R, Tai DS, Grody WW, Nguyen QG, Hauschka SD, Cederbaum SD, Lipshutz GS.

Mol Ther. 2014 Oct;22(10):1792-802. doi: 10.1038/mt.2014.99. Epub 2014 Jun 3.

10.

l-Citrulline Protects from Kidney Damage in Type 1 Diabetic Mice.

Romero MJ, Yao L, Sridhar S, Bhatta A, Dou H, Ramesh G, Brands MW, Pollock DM, Caldwell RB, Cederbaum SD, Head CA, Bagi Z, Lucas R, Caldwell RW.

Front Immunol. 2013 Dec 24;4:480. doi: 10.3389/fimmu.2013.00480. eCollection 2013.

11.

Analysis of cases of 3-methylcrotonyl CoA carboxylase deficiency (3-MCCD) in the California newborn screening program reported in the state database.

Lam C, Carter JM, Cederbaum SD, Neidich J, Gallant NM, Lorey F, Feuchtbaum L, Wong DA.

Mol Genet Metab. 2013 Dec;110(4):477-83. doi: 10.1016/j.ymgme.2013.09.006. Epub 2013 Sep 17.

PMID:
24103308
12.

Arginase 1: an unexpected mediator of pulmonary capillary barrier dysfunction in models of acute lung injury.

Lucas R, Czikora I, Sridhar S, Zemskov EA, Oseghale A, Circo S, Cederbaum SD, Chakraborty T, Fulton DJ, Caldwell RW, Romero MJ.

Front Immunol. 2013 Aug 7;4:228. doi: 10.3389/fimmu.2013.00228. eCollection 2013.

13.

Lethal phenotype in conditional late-onset arginase 1 deficiency in the mouse.

Kasten J, Hu C, Bhargava R, Park H, Tai D, Byrne JA, Marescau B, De Deyn PP, Schlichting L, Grody WW, Cederbaum SD, Lipshutz GS.

Mol Genet Metab. 2013 Nov;110(3):222-30. doi: 10.1016/j.ymgme.2013.06.020. Epub 2013 Jul 6.

14.

AAV-based gene therapy prevents neuropathology and results in normal cognitive development in the hyperargininemic mouse.

Lee EK, Hu C, Bhargava R, Ponnusamy R, Park H, Novicoff S, Rozengurt N, Marescau B, De Deyn P, Stout D, Schlichting L, Grody WW, Cederbaum SD, Lipshutz GS.

Gene Ther. 2013 Aug;20(8):785-96. doi: 10.1038/gt.2012.99. Epub 2013 Feb 7.

15.

Long-term survival of the juvenile lethal arginase-deficient mouse with AAV gene therapy.

Lee EK, Hu C, Bhargava R, Rozengurt N, Stout D, Grody WW, Cederbaum SD, Lipshutz GS.

Mol Ther. 2012 Oct;20(10):1844-51. doi: 10.1038/mt.2012.129. Epub 2012 Jul 3.

16.

Protein kinase C-α and arginase I mediate pneumolysin-induced pulmonary endothelial hyperpermeability.

Lucas R, Yang G, Gorshkov BA, Zemskov EA, Sridhar S, Umapathy NS, Jezierska-Drutel A, Alieva IB, Leustik M, Hossain H, Fischer B, Catravas JD, Verin AD, Pittet JF, Caldwell RB, Mitchell TJ, Cederbaum SD, Fulton DJ, Matthay MA, Caldwell RW, Romero MJ, Chakraborty T.

Am J Respir Cell Mol Biol. 2012 Oct;47(4):445-53. doi: 10.1165/rcmb.2011-0332OC. Epub 2012 May 10.

17.

Biochemical, molecular, and clinical characteristics of children with short chain acyl-CoA dehydrogenase deficiency detected by newborn screening in California.

Gallant NM, Leydiker K, Tang H, Feuchtbaum L, Lorey F, Puckett R, Deignan JL, Neidich J, Dorrani N, Chang E, Barshop BA, Cederbaum SD, Abdenur JE, Wang RY.

Mol Genet Metab. 2012 May;106(1):55-61. doi: 10.1016/j.ymgme.2012.02.007. Epub 2012 Feb 9.

PMID:
22424739
18.

Diabetes-induced vascular dysfunction involves arginase I.

Romero MJ, Iddings JA, Platt DH, Ali MI, Cederbaum SD, Stepp DW, Caldwell RB, Caldwell RW.

Am J Physiol Heart Circ Physiol. 2012 Jan 1;302(1):H159-66. doi: 10.1152/ajpheart.00774.2011. Epub 2011 Nov 4.

19.

Combined Hurler and Sanfilippo syndrome in a sibling pair.

Sun A, Hopwood JJ, Thompson J, Cederbaum SD.

Mol Genet Metab. 2011 Jun;103(2):135-7. doi: 10.1016/j.ymgme.2011.02.011. Epub 2011 Feb 22.

PMID:
21393040
20.

An exon 1 deletion in OTC identified using chromosomal microarray analysis in a mother and her two affected deceased newborns: implications for the prenatal diagnosis of ornithine transcarbamylase deficiency.

Quintero-Rivera F, Deignan JL, Peredo J, Grody WW, Crandall B, Sims M, Cederbaum SD.

Mol Genet Metab. 2010 Dec;101(4):413-6. doi: 10.1016/j.ymgme.2010.08.008. Epub 2010 Aug 14.

PMID:
20817516
21.

Guanidino compound levels in blood, cerebrospinal fluid, and post-mortem brain material of patients with argininemia.

Deignan JL, De Deyn PP, Cederbaum SD, Fuchshuber A, Roth B, Gsell W, Marescau B.

Mol Genet Metab. 2010;100 Suppl 1:S31-6. doi: 10.1016/j.ymgme.2010.01.012. Epub 2010 Jan 29.

PMID:
20176499
22.

Diffusion tensor imaging in arginase deficiency reveals damage to corticospinal tracts.

Oldham MS, VanMeter JW, Shattuck KF, Cederbaum SD, Gropman AL.

Pediatr Neurol. 2010 Jan;42(1):49-52. doi: 10.1016/j.pediatrneurol.2009.07.017.

23.

Bone marrow cell derived arginase I is the major source of allergen-induced lung arginase but is not required for airway hyperresponsiveness, remodeling and lung inflammatory responses in mice.

Niese KA, Collier AR, Hajek AR, Cederbaum SD, O'Brien WE, Wills-Karp M, Rothenberg ME, Zimmermann N.

BMC Immunol. 2009 Jun 1;10:33. doi: 10.1186/1471-2172-10-33.

24.

Short-term correction of arginase deficiency in a neonatal murine model with a helper-dependent adenoviral vector.

Gau CL, Rosenblatt RA, Cerullo V, Lay FD, Dow AC, Livesay J, Brunetti-Pierri N, Lee B, Cederbaum SD, Grody WW, Lipshutz GS.

Mol Ther. 2009 Jul;17(7):1155-63. doi: 10.1038/mt.2009.65. Epub 2009 Apr 14.

25.

Disruption of arginase II alters prostate tumor formation in TRAMP mice.

Mumenthaler SM, Rozengurt N, Livesay JC, Sabaghian A, Cederbaum SD, Grody WW.

Prostate. 2008 Oct 1;68(14):1561-9. doi: 10.1002/pros.20816.

PMID:
18663728
26.

Cross-sectional multicenter study of patients with urea cycle disorders in the United States.

Tuchman M, Lee B, Lichter-Konecki U, Summar ML, Yudkoff M, Cederbaum SD, Kerr DS, Diaz GA, Seashore MR, Lee HS, McCarter RJ, Krischer JP, Batshaw ML; Urea Cycle Disorders Consortium of the Rare Diseases Clinical Research Network.

Mol Genet Metab. 2008 Aug;94(4):397-402. doi: 10.1016/j.ymgme.2008.05.004. Epub 2008 Jun 17.

27.

Maternal glutaric acidemia, type I identified by newborn screening.

Crombez EA, Cederbaum SD, Spector E, Chan E, Salazar D, Neidich J, Goodman S.

Mol Genet Metab. 2008 May;94(1):132-4. doi: 10.1016/j.ymgme.2008.01.005. Epub 2008 Mar 4.

28.

Expression of arginase II in prostate cancer.

Mumenthaler SM, Yu H, Tze S, Cederbaum SD, Pegg AE, Seligson DB, Grody WW.

Int J Oncol. 2008 Feb;32(2):357-65.

PMID:
18202758
29.

Increased plasma and tissue guanidino compounds in a mouse model of hyperargininemia.

Deignan JL, Marescau B, Livesay JC, Iyer RK, De Deyn PP, Cederbaum SD, Grody WW.

Mol Genet Metab. 2008 Feb;93(2):172-8. Epub 2007 Nov 7.

PMID:
17997338
30.

Contrasting features of urea cycle disorders in human patients and knockout mouse models.

Deignan JL, Cederbaum SD, Grody WW.

Mol Genet Metab. 2008 Jan;93(1):7-14. Epub 2007 Oct 22. Review.

31.

Polyamine homeostasis in arginase knockout mice.

Deignan JL, Livesay JC, Shantz LM, Pegg AE, O'Brien WE, Iyer RK, Cederbaum SD, Grody WW.

Am J Physiol Cell Physiol. 2007 Oct;293(4):C1296-301. Epub 2007 Aug 8.

32.
33.

Clinical and functional characterization of a human ORNT1 mutation (T32R) in the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome.

Camacho JA, Mardach R, Rioseco-Camacho N, Ruiz-Pesini E, Derbeneva O, Andrade D, Zaldivar F, Qu Y, Cederbaum SD.

Pediatr Res. 2006 Oct;60(4):423-9. Epub 2006 Aug 28.

PMID:
16940241
34.

Arginase induction by sodium phenylbutyrate in mouse tissues and human cell lines.

Kern RM, Yang Z, Kim PS, Grody WW, Iyer RK, Cederbaum SD.

Mol Genet Metab. 2007 Jan;90(1):37-41. Epub 2006 Aug 28.

PMID:
16935537
35.

Loss of arginase I results in increased proliferation of neural stem cells.

Becker-Catania SG, Gregory TL, Yang Y, Gau CL, de Vellis J, Cederbaum SD, Iyer RK.

J Neurosci Res. 2006 Sep;84(4):735-46.

PMID:
16773651
36.

Ornithine deficiency in the arginase double knockout mouse.

Deignan JL, Livesay JC, Yoo PK, Goodman SI, O'Brien WE, Iyer RK, Cederbaum SD, Grody WW.

Mol Genet Metab. 2006 Sep-Oct;89(1-2):87-96. Epub 2006 Jun 5.

PMID:
16753325
37.

New frontiers in hereditary metabolic disease: an historical perspective.

Cederbaum SD.

Mol Genet Metab. 2006 Mar;87(3):184-9. Epub 2006 Jan 5. No abstract available.

PMID:
16403665
38.

Clinical, pathological, and biochemical studies in a patient with propionic acidemia and fatal cardiomyopathy.

Mardach R, Verity MA, Cederbaum SD.

Mol Genet Metab. 2005 Aug;85(4):286-90.

PMID:
15939644
39.

Hyperargininemia due to liver arginase deficiency.

Crombez EA, Cederbaum SD.

Mol Genet Metab. 2005 Mar;84(3):243-51. Epub 2004 Dec 19. Review.

PMID:
15694174
40.

Arginases I and II: do their functions overlap?

Cederbaum SD, Yu H, Grody WW, Kern RM, Yoo P, Iyer RK.

Mol Genet Metab. 2004 Apr;81 Suppl 1:S38-44. Review.

PMID:
15050972
41.

Widespread expression of arginase I in mouse tissues. Biochemical and physiological implications.

Yu H, Yoo PK, Aguirre CC, Tsoa RW, Kern RM, Grody WW, Cederbaum SD, Iyer RK.

J Histochem Cytochem. 2003 Sep;51(9):1151-60.

PMID:
12923240
42.

Arginase deficiency with lethal neonatal expression: evidence for the glutamine hypothesis of cerebral edema.

Picker JD, Puga AC, Levy HL, Marsden D, Shih VE, Degirolami U, Ligon KL, Cederbaum SD, Kern RM, Cox GF.

J Pediatr. 2003 Mar;142(3):349-52.

PMID:
12640389
43.

Carnitine membrane transporter deficiency: a long-term follow up and OCTN2 mutation in the first documented case of primary carnitine deficiency.

Cederbaum SD, Koo-McCoy S, Tein I, Hsu BY, Ganguly A, Vilain E, Dipple K, Cvitanovic-Sojat L, Stanley C.

Mol Genet Metab. 2002 Nov;77(3):195-201. Erratum in: Mol Genet Metab. 2003 Jan;78(1):82.

PMID:
12409266
44.

Identification of isobutyryl-CoA dehydrogenase and its deficiency in humans.

Nguyen TV, Andresen BS, Corydon TJ, Ghisla S, Abd-El Razik N, Mohsen AW, Cederbaum SD, Roe DS, Roe CR, Lench NJ, Vockley J.

Mol Genet Metab. 2002 Sep-Oct;77(1-2):68-79.

PMID:
12359132
45.

Expression of the liver form of arginase in erythrocytes.

Kim PS, Iyer RK, Lu KV, Yu H, Karimi A, Kern RM, Tai DK, Cederbaum SD, Grody WW.

Mol Genet Metab. 2002 Jun;76(2):100-10.

PMID:
12083807
46.

Mouse model for human arginase deficiency.

Iyer RK, Yoo PK, Kern RM, Rozengurt N, Tsoa R, O'Brien WE, Yu H, Grody WW, Cederbaum SD.

Mol Cell Biol. 2002 Jul;22(13):4491-8.

47.

Arginase expression in mouse embryonic development.

Yu H, Iyer RK, Yoo PK, Kern RM, Grody WW, Cederbaum SD.

Mech Dev. 2002 Jul;115(1-2):151-5.

48.

Helicobacter pylori induces macrophage apoptosis by activation of arginase II.

Gobert AP, Cheng Y, Wang JY, Boucher JL, Iyer RK, Cederbaum SD, Casero RA Jr, Newton JC, Wilson KT.

J Immunol. 2002 May 1;168(9):4692-700.

49.

Cloning and characterization of human agmatinase.

Iyer RK, Kim HK, Tsoa RW, Grody WW, Cederbaum SD.

Mol Genet Metab. 2002 Mar;75(3):209-18.

PMID:
11914032
50.

Expression of arginase isozymes in mouse brain.

Yu H, Iyer RK, Kern RM, Rodriguez WI, Grody WW, Cederbaum SD.

J Neurosci Res. 2001 Nov 1;66(3):406-22.

PMID:
11746358

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