Stacpoole PW - Search Results

1

Phenylbutyrate therapy for pyruvate dehydrogenase complex deficiency and lactic acidosis.

Ferriero R, Manco G, Lamantea E, Nusco E, Ferrante MI, Sordino P, Stacpoole PW, Lee B, Zeviani M, Brunetti-Pierri N. Ferriero R, et al. Among authors: stacpoole pw. Sci Transl Med. 2013 Mar 6;5(175):175ra31. doi: 10.1126/scitranslmed.3004986. Sci Transl Med. 2013. PMID: 23467562 Free PMC article.

2

Pyruvate dehydrogenase complex deficiency caused by ubiquitination and proteasome-mediated degradation of the E1 subunit.

Han Z, Zhong L, Srivastava A, Stacpoole PW. Han Z, et al. Among authors: stacpoole pw. J Biol Chem. 2008 Jan 4;283(1):237-243. doi: 10.1074/jbc.M704748200. Epub 2007 Oct 8. J Biol Chem. 2008. PMID: 17923481 Free article.

3

The spectrum of pyruvate dehydrogenase complex deficiency: clinical, biochemical and genetic features in 371 patients.

Patel KP, O'Brien TW, Subramony SH, Shuster J, Stacpoole PW. Patel KP, et al. Among authors: stacpoole pw. Mol Genet Metab. 2012 Jul;106(3):385-94. doi: 10.1016/j.ymgme.2012.03.017. Mol Genet Metab. 2012. PMID: 22896851 Free PMC article.

4

Therapeutic potential of dichloroacetate for pyruvate dehydrogenase complex deficiency.

Berendzen K, Theriaque DW, Shuster J, Stacpoole PW. Berendzen K, et al. Among authors: stacpoole pw. Mitochondrion. 2006 Jun;6(3):126-35. doi: 10.1016/j.mito.2006.04.001. Epub 2006 May 3. Mitochondrion. 2006. PMID: 16725381

5

The spectrum of pyruvate dehydrogenase complex deficiency: clinical, biochemical and genetic features in 371 patients.

Patel KP, O'Brien TW, Subramony SH, Shuster J, Stacpoole PW. Patel KP, et al. Among authors: stacpoole pw. Mol Genet Metab. 2012 Jan;105(1):34-43. doi: 10.1016/j.ymgme.2011.09.032. Epub 2011 Oct 7. Mol Genet Metab. 2012. Corrected and republished in: Mol Genet Metab. 2012 Jul;106(3):385-94. doi: 10.1016/j.ymgme.2012.03.017. PMID: 22079328 Free PMC article. Corrected and republished. Review.

6

The pyruvate dehydrogenase complex as a target for gene therapy.

Stacpoole PW, Owen R, Flotte TR. Stacpoole PW, et al. Curr Gene Ther. 2003 Jun;3(3):239-45. doi: 10.2174/1566523034578320. Curr Gene Ther. 2003. PMID: 12762482 Review.

7

Lactic acidosis.

Stacpoole PW. Stacpoole PW. Endocrinol Metab Clin North Am. 1993 Jun;22(2):221-45. Endocrinol Metab Clin North Am. 1993. PMID: 8325284 Review.

8

Renal manifestations of congenital lactic acidosis.

Neiberger RE, George JC, Perkins LA, Theriaque DW, Hutson AD, Stacpoole PW. Neiberger RE, et al. Among authors: stacpoole pw. Am J Kidney Dis. 2002 Jan;39(1):12-23. doi: 10.1053/ajkd.2002.29872. Am J Kidney Dis. 2002. PMID: 11774096

9

Magnetic resonance spectroscopic investigation of mitochondrial fuel metabolism and energetics in cultured human fibroblasts: effects of pyruvate dehydrogenase complex deficiency and dichloroacetate.

Simpson NE, Han Z, Berendzen KM, Sweeney CA, Oca-Cossio JA, Constantinidis I, Stacpoole PW. Simpson NE, et al. Among authors: stacpoole pw. Mol Genet Metab. 2006 Sep-Oct;89(1-2):97-105. doi: 10.1016/j.ymgme.2006.04.015. Epub 2006 Jun 12. Mol Genet Metab. 2006. PMID: 16765624

10

Defects in the E2 lipoyl transacetylase and the X-lipoyl containing component of the pyruvate dehydrogenase complex in patients with lactic acidemia.

Robinson BH, MacKay N, Petrova-Benedict R, Ozalp I, Coskun T, Stacpoole PW. Robinson BH, et al. Among authors: stacpoole pw. J Clin Invest. 1990 Jun;85(6):1821-4. doi: 10.1172/JCI114641. J Clin Invest. 1990. PMID: 2112155 Free PMC article.

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