Paul HS - Search Results

1

Complementation of defective leucine decarboxylation in fibroblasts from a maple syrup urine disease patient by retrovirus-mediated gene transfer.

Mueller GM, McKenzie LR, Homanics GE, Watkins SC, Robbins PD, Paul HS. Mueller GM, et al. Among authors: paul hs. Gene Ther. 1995 Sep;2(7):461-8. Gene Ther. 1995. PMID: 7584124

2

Production and characterization of murine models of classic and intermediate maple syrup urine disease.

Homanics GE, Skvorak K, Ferguson C, Watkins S, Paul HS. Homanics GE, et al. Among authors: paul hs. BMC Med Genet. 2006 Mar 31;7:33. doi: 10.1186/1471-2350-7-33. BMC Med Genet. 2006. PMID: 16579849 Free PMC article.

3

Hepatocyte transplantation improves phenotype and extends survival in a murine model of intermediate maple syrup urine disease.

Skvorak KJ, Paul HS, Dorko K, Marongiu F, Ellis E, Chace D, Ferguson C, Gibson KM, Homanics GE, Strom SC. Skvorak KJ, et al. Among authors: paul hs. Mol Ther. 2009 Jul;17(7):1266-73. doi: 10.1038/mt.2009.99. Epub 2009 May 12. Mol Ther. 2009. PMID: 19436271 Free PMC article.

4

Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease.

Zinnanti WJ, Lazovic J, Griffin K, Skvorak KJ, Paul HS, Homanics GE, Bewley MC, Cheng KC, Lanoue KF, Flanagan JM. Zinnanti WJ, et al. Among authors: paul hs. Brain. 2009 Apr;132(Pt 4):903-18. doi: 10.1093/brain/awp024. Epub 2009 Mar 17. Brain. 2009. PMID: 19293241 Free PMC article.

5

Hepatocyte transplantation (HTx) corrects selected neurometabolic abnormalities in murine intermediate maple syrup urine disease (iMSUD).

Skvorak KJ, Hager EJ, Arning E, Bottiglieri T, Paul HS, Strom SC, Homanics GE, Sun Q, Jansen EE, Jakobs C, Zinnanti WJ, Gibson KM. Skvorak KJ, et al. Among authors: paul hs. Biochim Biophys Acta. 2009 Oct;1792(10):1004-10. doi: 10.1016/j.bbadis.2009.08.006. Epub 2009 Aug 19. Biochim Biophys Acta. 2009. PMID: 19699299 Free PMC article.

6

Posttranscriptional alterations in protein masses of hepatic branched-chain keto acid dehydrogenase and its associated kinase in diabetes.

Lombardo YB, Thamotharan M, Bawani SZ, Paul HS, Adibi SA. Lombardo YB, et al. Among authors: paul hs. Proc Assoc Am Physicians. 1998 Jan-Feb;110(1):40-9. Proc Assoc Am Physicians. 1998. PMID: 9460082

7

Alteration in gene expression of branched-chain keto acid dehydrogenase kinase but not in gene expression of its substrate in the liver of clofibrate-treated rats.

Paul HS, Liu WQ, Adibi SA. Paul HS, et al. Biochem J. 1996 Jul 15;317 ( Pt 2)(Pt 2):411-7. doi: 10.1042/bj3170411. Biochem J. 1996. PMID: 8713066 Free PMC article.

8

Regulation of gene expression of branched-chain keto acid dehydrogenase complex in primary cultured hepatocytes by dexamethasone and a cAMP analog.

Chicco AG, Adibi SA, Liu WQ, Morris SM Jr, Paul HS. Chicco AG, et al. Among authors: paul hs. J Biol Chem. 1994 Jul 29;269(30):19427-34. J Biol Chem. 1994. PMID: 8034710 Free article.

9

Investigation of the presence of branched-chain alpha-keto acid dehydrogenase in mammalian hepatic peroxisomes.

Paul HS, Sekas G, Adibi SA. Paul HS, et al. Int J Biochem. 1992 Apr;24(4):617-9. doi: 10.1016/0020-711x(92)90336-y. Int J Biochem. 1992. PMID: 1516734

10

Mechanism of increased conversion of branched chain keto acid dehydrogenase from inactive to active form by a medium chain fatty acid (octanoate) in skeletal muscle.

Paul HS, Adibi SA. Paul HS, et al. J Biol Chem. 1992 Jun 5;267(16):11208-14. J Biol Chem. 1992. PMID: 1597456 Free article.

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