Search Results - "Firth, Stephen D"

The directed differentiation of human iPS cells into kidney podocytes by Song, Bi, Smink, Alexandra M, Jones, Christina V, Callaghan, Judy M, Firth, Stephen D, Bernard, Claude A, Laslett, Andrew L, Kerr, Peter G, Ricardo, Sharon D

“…The loss of glomerular podocytes is a key event in the progression of chronic kidney disease resulting in proteinuria and declining function. Podocytes are…”
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Validation of a Three-Dimensional Method for Counting and Sizing Podocytes in Whole Glomeruli by Puelles, Victor G, van der Wolde, James W, Schulze, Keith E, Short, Kieran M, Wong, Milagros N, Bensley, Jonathan G, Cullen-McEwen, Luise A, Caruana, Georgina, Hokke, Stacey N, Li, Jinhua, Firth, Stephen D, Harper, Ian S, Nikolic-Paterson, David J, Bertram, John F

“…Podocyte depletion is sufficient for the development of numerous glomerular diseases and can be absolute (loss of podocytes) or relative (reduced number of…”
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Copper-transporting P-type ATPase, ATP7A, confers multidrug resistance and its expression is related to resistance to SN-38 in clinical colon cancer by OWATARI, Satsuki, AKUNE, Satoshi, TAKEUCHI, Toru, AIKOU, Takashi, MERCER, Julian F. B, AKIYAMA, Shin-Ichi, FURUKAWA, Tatsuhiko, KOMATSU, Masaharu, IKEDA, Ryuji, FIRTH, Stephen D, CHE, Xiao-Fang, YAMAMOTO, Masatatsu, TSUJIKAWA, Kazutake, KITAZONO, Masaki, ISHIZAWA, Takashi

“…We and others have shown that the copper transporters ATP7A and ATP7B play a role in cellular resistance to cis-diaminedichloroplatinum (II) (CDDP). In this…”
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Kinetic Analysis of the Interaction of the Copper Chaperone Atox1 with the Metal Binding Sites of the Menkes Protein by Strausak, Daniel, Howie, Michelle K., Firth, Stephen D., Schlicksupp, Andrea, Pipkorn, Rüdiger, Multhaup, Gerd, Mercer, Julian F.B.

“…Excess copper is effluxed from mammalian cells by the Menkes or Wilson P-type ATPases (MNK and WND, respectively). MNK and WND have six metal binding sites…”
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The Role of GMXCXXC Metal Binding Sites in the Copper-induced Redistribution of the Menkes Protein by Strausak, D, La Fontaine, S, Hill, J, Firth, S D, Lockhart, P J, Mercer, J F

“…The Menkes protein (MNK or ATP7A) is a transmembrane, copper-transporting CP X -type ATPase, a subgroup of the extensive family of P-type ATPases. A striking…”
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Effect of the toxic milk mutation (tx) on the function and intracellular localization of Wnd, the murine homologue of the Wilson copper ATPase by La Fontaine, S, Theophilos, M B, Firth, S D, Gould, R, Parton, R G, Mercer, J F

“…Wilson disease is an autosomal recessive copper transport disorder resulting from defective biliary excretion of copper and subsequent hepatic copper…”
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Correction of the Copper Transport Defect of Menkes Patient Fibroblasts by Expression of the Menkes and Wilson ATPases by Fontaine, Sharon La, Firth, Stephen D., Camakaris, James, Englezou, Anna, Theophilos, Michael B., Petris, Michael J., Howie, Michelle, Lockhart, Paul J., Greenough, Mark, Brooks, Hilary, Reddel, Roger R., Mercer, Julian F.B.

“…Menkes' disease is a fatal, X-linked, copper deficiency disorder that results from defective copper efflux from intestinal cells and inadequate copper delivery…”
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Correction of the copper transport defect of Menkes patient fibroblasts by expression of two forms of the sheep Wilson ATPase by Lockhart, Paul J, La Fontaine, Sharon, Firth, Stephen D, Greenough, Mark, Camakaris, James, Mercer, Julian F.B

“…The Wilson disease (WD) protein (ATP7B) is a copper-transporting P-type ATPase that is responsible for the efflux of hepatic copper into the bile, a process…”
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Effect of the toxic milk mutation (tx) on the function and intracellular localization of Wnd, the murine homologue of the Wilson copperATPase by LA FONTAINE, Sharon, THEOPHILOS, Michael B, FIRTH, Stephen D, GOULD, Robert, PARTON, Robert G, MERCER, Julian F. B

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Functional Analysis and Intracellular Localization of the Human Menkes Protein (MNK) Stably Expressed from a cDNA Construct in Chinese Hamster Ovary Cells (CHO-K1) by La Fontaine, Sharon, Firth, Stephen D., Lockhart, Paul J., Brooks, Hilary, Parton, Robert G., Camakaris, James, Mercer, Julian F. B.

“…The Menkes protein (MNK or ATP7A) is an important component of the mammalian copper transport pathway and is defective in Menkes disease, a fatal X-linked…”
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Intracellular Localization and Loss of Copper Responsiveness of Mnk, the Murine Homologue of the Menkes Protein, in Cells from Blotchy (Moblo) and Brindled (Mobr) Mouse Mutants by La Fontaine, Sharon, Firth, Stephen D., Lockhart, Paul J., Brooks, Hilary, Camakaris, James, Mercer, Julian F. B.

“…Menkes disease is an X-linked copper deficiency disorder that results from mutations in the ATP7A (MNK) gene. A wide range of disease-causing mutations within…”
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Functional analysis of the Menkes protein (MNK) expressed from a cDNA construct by La Fontaine, S, Firth, S D, Lockhart, P J, Brooks, H, Camakaris, J, Mercer, J F

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Eukaryotic Expression Vectors That Replicate to Low Copy Number in Bacteria: Transient Expression of the Menkes Protein by Fontaine, Sharon La, Firth, Stephen D., Lockhart, Paul J., Paynter, Jennifer A., Mercer, Julian F.B.

“…A set of low copy number plasmid vectors for mammalian gene expression has been constructed. These vectors are derived from the previously described bacterial…”
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