Search Results - "Fenton, W. A."

GroEL‐Mediated protein folding by Fenton, Wayne A., Horwich, Arthur L.

“…I Architecture of GroEL and GroES and the reaction pathway A. Architecture of the chaperonins B. Reaction pathway of GroEL‐GroES‐mediated folding II…”
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Residues in chaperonin GroEL required for polypeptide binding and release by Fenton, Wayne A, Kashi, Yechezkel, Furtak, Krystyna, Norwich, Arthur L

“…Chaperonins are ring-shaped protein complexes that are essential in the cell, mediating ATP-dependent polypeptide folding in a variety of compartments. Recent…”
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Clinical heterogeneity and prognosis in combined methylmalonic aciduria and homocystinuria (cblC) by Rosenblatt, D. S., Aspler, A. L., Shevell, M. I., Pletcher, B. A., Fenton, W. A., Seashore, M. R.

“…The clbC form of methylmalonic acidaemia is a rare and poorly understood condition which results from impaired biosynthesis of methylcobalamin and…”
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Distinct actions of cis and trans ATP within the double ring of the chaperonin GroEL by Rye, Hays S, Burston, Steven G, Fenton, Wayne A, Beechem, Joseph M, Xu, Zhaohui, Sigler, Paul B, Horwich, Arthur L

“…The chaperonin GroEL is a double-ring structure with a central cavity in each ring that provides an environment for the efficient folding of proteins when…”
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Folding in vivo of bacterial cytoplasmic proteins: role of GroEL by Horwich, A L, Low, K B, Fenton, W A, Hirshfield, I N, Furtak, K

“…A general role for chaperonin ring structures in mediating folding of newly translated proteins has been suggested. Here we have directly examined the role of…”
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Release of both native and non-native proteins from a cis -only GroEL ternary complex by Burston, Steven G, Weissman, Jonathan S, Farr, George W, Fenton, Wayne A, Norwich, Arthur L

“…Protein folding by the double-ring chaperonin GroEL is initiated in cis ternary complexes, in which polypeptide is sequestered in the central channel of a…”
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Stable transformation of CHO Cells and human NARP cybrids confers oligomycin resistance (oli(r)) following transfer of a mitochondrial DNA-encoded oli(r) ATPase6 gene to the nuclear genome: a model system for mtDNA gene therapy by Zullo, S J, Parks, W T, Chloupkova, M, Wei, B, Weiner, H, Fenton, W A, Eisenstadt, J M, Merril, C R

“…Point and deletion mutations and a general depletion of mammalian mitochondrial DNA (mtDNA) give rise to a wide variety of medical syndromes that are…”
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Cleavage of Precursors by the Mitochondrial Processing Peptidase Requires a Compatible Mature Protein or an Intermediate Octapeptide by Isaya, Grazia, Kalousek, Frantisek, Fenton, Wayne A., Rosenberg, Leon E.

“…Many precursors of mitochondrial proteins are processed in two successive steps by independent matrix peptidases (MPP and MIP), whereas others are cleaved in a…”
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Expression and Kinetic Characterization of Methylmalonyl-CoA Mutase from Patients with the Mut− Phenotype: Evidence for Naturally Occurring Interallelic Complementation by Janata, Jirί, Kogekar, Nandini, Fenton, Wayne A.

“…l-Methylmalonyl-CoA mutase (MUT) is an adenosylcobalamin (AdoCbl)-requiring mitochondrial matrix enzyme that catalyzes the isomerization of l-methylmalonyl-CoA…”
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Citrate inhibition of snake venom proteases by Odell, G.V, Ferry, P.C, Vick, L.M, Fenton, A.W, Decker, L.S, Cowell, R.L, Ownby, C.L, Gutierrez, J.M

“…Thirty snake venoms had a citrate content of 2.3 to 12.9%, dry basis, by an aconitase–isocitric dehydrogenase coupled enzyme assay. This is a venom…”
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GroEL, GroES, and ATP-dependent folding and spontaneous assembly of ornithine transcarbamylase by XIANXIAN ZHENG, ROSENBERG, L. E, KALOUSEK, F, FENTON, W. A

“…When purified rat liver ornithine transcarbamylase (OTC), a trimer of 36 kDa subunits, was denatured in 6 M guanidine hydrochloride and then diluted…”
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Mitochondrial protein transport--a system in search of mutations by Fenton, W A

“…After years of searching in the cDNA of patients with defects in specific mitochondrial proteins and, with somewhat less diligence, in patients with undefined…”
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The venomous hair structure, venom and life cycle of Lagoa crispata, a puss caterpillar of Oklahoma by Lamdin, J.M., Howell, D.E., Kocan, K.M., Murphey, D.R., Arnold, D.C., Fenton, A.W., Odell, G.V., Ownby, C.L.

“…The presence of a unique population of Lagoa crispata, puss caterpillar, in western Oklahoma is reported. A detailed microscopic examination shows the…”
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The β Subunit of the Mitochondrial Processing Peptidase From Rat Liver: Cloning and Sequencing of a cDNA and Comparison With a Proposed Family of Metallopeptidases by Paces, Vaclav, Rosenberg, Leon E., Fenton, Wayne A., Kalousek, Frantisek

“…Most nuclearly encoded mitochondrial proteins are synthesized with amino-terminal leader peptides that are removed by the mitochondrial processing peptidase…”
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ATP-Bound States of GroEL Captured by Cryo-Electron Microscopy by Ranson, Neil A., Farr, George W., Roseman, Alan M., Gowen, Brent, Fenton, Wayne A., Horwich, Arthur L., Saibil, Helen R.

“…The chaperonin GroEL drives its protein-folding cycle by cooperatively binding ATP to one of its two rings, priming that ring to become folding-active upon…”
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Orthotopic liver transplantation for urea cycle enzyme deficiency by Todo, S, Starzl, T E, Tzakis, A, Benkov, K J, Kalousek, F, Saheki, T, Tanikawa, K, Fenton, W A

“…Hyperammonemia, abnormalities in plasma amino acids and abnormalities of standard liver functions were corrected by orthotopic liver transplantation in a…”
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GroEL-GroES Cycling: ATP and Nonnative Polypeptide Direct Alternation of Folding-Active Rings by Rye, Hays S., Roseman, Alan M., Chen, Shaoxia, Furtak, Krystyna, Fenton, Wayne A., Saibil, Helen R., Horwich, Arthur L.

“…The double-ring chaperonin GroEL mediates protein folding in the central cavity of a ring bound by ATP and GroES, but it is unclear how GroEL cycles from one…”
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GroEL/GroES-Mediated Folding of a Protein Too Large to Be Encapsulated by Chaudhuri, Tapan K., Farr, George W., Fenton, Wayne A., Rospert, Sabine, Horwich, Arthur L.

“…The chaperonin GroEL binds nonnative proteins too large to fit inside the productive GroEL-GroES cis cavity, but whether and how it assists their folding has…”
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STRUCTURE AND FUNCTION IN GroEL-MEDIATED PROTEIN FOLDING by Sigler, Paul B, Xu, Zhaohui, Rye, Hays S, Burston, Steven G, Fenton, Wayne A, Horwich, Arthur L

“…Recent structural and biochemical investigations have come together to allow a better understanding of the mechanism of chaperonin (GroEL, Hsp60)-mediated…”
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Identification of RNA splicing errors resulting in human ornithine transcarbamylase deficiency by CARSTENS, R. P, FENTON, W. A, ROSENBERG, L. R

“…Ornithine transcarbamylase (OTC) is an X-linked, liver-specific enzyme that catalyzes the second step of the urea cycle. In humans, inherited deficiency of OTC…”
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