Search Results - "Edmunds, Tim"

Human acid sphingomyelinase structures provide insight to molecular basis of Niemann–Pick disease by Zhou, Yan-Feng, Metcalf, Matthew C., Garman, Scott C., Edmunds, Tim, Qiu, Huawei, Wei, Ronnie R.

“…Acid sphingomyelinase (ASM) hydrolyzes sphingomyelin to ceramide and phosphocholine, essential components of myelin in neurons. Genetic alterations in ASM lead…”
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Emerging therapeutic strategies for the treatment of fabry disease by Edmunds, Tim, PhD

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Identification and quantitation of vesivirus 2117 particles in bioreactor fluids from infected Chinese hamster ovary cell cultures by Qiu, Yongchang, Jones, Nathan, Busch, Michelle, Pan, Peng, Keegan, Jesse, Zhou, Weichang, Plavsic, Mark, Hayes, Michael, McPherson, John M., Edmunds, Tim, Zhang, Kate, Mattaliano, Robert J.

“…The prevention of adventitious agent contamination is a top priority throughout the entire biopharmaceutical production process. For example, although viral…”
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Effect of mannose chain length on targeting of glucocerebrosidase for enzyme replacement therapy of Gaucher disease by Van Patten, Scott M, Hughes, Heather, Huff, Michael R, Piepenhagen, Peter A, Waire, James, Qiu, Huawei, Ganesa, Chandrashekar, Reczek, David, Ward, Paul V, Kutzko, Joseph P, Edmunds, Tim

“…Recombinant human glucocerebrosidase (imiglucerase, Cerezyme®) is used in enzyme replacement therapy for Gaucher disease. Complex oligosaccharides present on…”
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Designing Protein Therapies: Lessons Learned from Cerezyme® for Gaucher Disease by Edmunds, Tim, PhD

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Activation of Human Acid Sphingomyelinase through Modification or Deletion of C-terminal Cysteine by Qiu, Huawei, Edmunds, Tim, Baker-Malcolm, Jennifer, Karey, Kenneth P., Estes, Scott, Schwarz, Cordula, Hughes, Heather, Van Patten, Scott M.

“…One form of Niemann-Pick disease is caused by a deficiency in the enzymatic activity of acid sphingomyelinase. During efforts to develop an enzyme replacement…”
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Structural and biochemical studies on Pompe disease and a "pseudodeficiency of acid α-glucosidase" by Tajima, Youichi, Matsuzawa, Fumiko, Aikawa, Sei-Ichi, Okumiya, Toshika, Yoshimizu, Michiru, Tsukimura, Takahiro, Ikekita, Masahiko, Tsujino, Seiichi, Tsuji, Akihiko, Edmunds, Tim, Sakuraba, Hitoshi

“…We constructed structural models of the catalytic domain and the surrounding region of human wild-type acid alpha-glucosidase and the enzyme with amino acid…”
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Effect of genetic background on glycosylation heterogeneity in human antithrombin produced in the mammary gland of transgenic goats by Zhou, Qun, Kyazike, Josephine, Echelard, Yann, Meade, Harry M., Higgins, Elizabeth, Cole, Edward S., Edmunds, Tim

“…Glycosylation is involved in the correct folding, targeting, bioactivity and clearance of therapeutic glycoproteins. With the development of transgenic animals…”
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Transgenically Produced Human Antithrombin: Structural and Functional Comparison to Human Plasma–Derived Antithrombin by Edmunds, Tim, Van Patten, Scott M., Pollock, Julie, Hanson, Eric, Bernasconi, Richard, Higgins, Elizabeth, Manavalan, Partha, Ziomek, Carol, Meade, Harry, McPherson, John M., Cole, Edward S.

“…Recombinant human antithrombin (rhAT) produced in transgenic goat milk was purified to greater than 99%. The specific activity of the rhAT was identical to…”
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LIMP-2 Is a Receptor for Lysosomal Mannose-6-Phosphate-Independent Targeting of β-Glucocerebrosidase by Reczek, David, Schwake, Michael, Schröder, Jenny, Hughes, Heather, Blanz, Judith, Jin, Xiaoying, Brondyk, William, Van Patten, Scott, Edmunds, Tim, Saftig, Paul

“…β-glucocerebrosidase, the enzyme defective in Gaucher disease, is targeted to the lysosome independently of the mannose-6-phosphate receptor…”
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Chemical Chaperones and Permissive Temperatures Alter the Cellular Localization of Gaucher Disease Associated Glucocerebrosidase Variants by Sawkar, Anu R, Schmitz, Martina, Zimmer, Klaus-Peter, Reczek, David, Edmunds, Tim, Balch, William E, Kelly, Jeffery W

“…Point mutations in the lysosomal hydrolase, glucocerebrosidase (GC), can cause Gaucher disease, a common lysosomal storage disease. Several clinically…”
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Militarization, stigma, and resistance: negotiating military reservist identity in the civilian workplace by Higate, Paul, Dawes, Antonia, Edmunds, Tim, Jenkings, K. Neil, Woodward, Rachel

“…Set against the backdrop of the British Government's Future Reserves 2020 (FR2020) programme, this article addresses military reservists' experiences of how…”
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Development of a simple and rapid method for producing non-fucosylated oligomannose containing antibodies with increased effector function by Zhou, Qun, Shankara, Srinivas, Roy, Andre, Qiu, Huawei, Estes, Scott, McVie-Wylie, Alison, Culm-Merdek, Kerry, Park, Anna, Pan, Clark, Edmunds, Tim

“…Glycosylation in the Fc region of antibodies has been shown to play an important role in antibody function. In the current study, glycosylation of human…”
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Antithrombin−Heparin Affinity Reduced by Fucosylation of Carbohydrate at Asparagine 155 by Garone, Louise, Edmunds, Tim, Hanson, Eric, Bernasconi, Richard, Huntington, James A, Meagher, Jennifer L, Fan, Bingqi, Gettins, Peter G. W

“…The two human plasma antithrombin isoforms, α and β, differ in glycosylation at asparagine 135. Only the α form carries carbohydrate at this position and has…”
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Recombinant Human Thyroid Stimulating Hormone: Development of a Biotechnology Product for Detection of Metastatic Lesions of Thyroid Carcinoma by Cole, Edward S, Lee, Karen, Lauziere, Kevin, Kelton, Christine, Chappel, Scott, Weintraub, Bruce, Ferrara, Dawn, Peterson, Pamela, Bernasconi, Rick, Edmunds, Tim, Richards, Susan, Dickrell, Lydia, Kleeman, James M, McPherson, John M, Pratt, Bruce M

“…We have genetically engineered a cell line, and developed a reproducible process, for the expression and purification of biologically active recombinant human…”
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Impact of cysteine variants on the structure, activity, and stability of recombinant human α‐galactosidase A by Qiu, Huawei, Honey, Denise M., Kingsbury, Jonathan S., Park, Anna, Boudanova, Ekaterina, Wei, Ronnie R., Pan, Clark Q., Edmunds, Tim

“…Recombinant human α‐galactosidase A (rhαGal) is a homodimeric glycoprotein deficient in Fabry disease, a lysosomal storage disorder. In this study, each…”
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X-ray and Biochemical Analysis of N370S Mutant Human Acid β-Glucosidase by Wei, Ronnie R., Hughes, Heather, Boucher, Susan, Bird, Julie J., Guziewicz, Nicholas, Van Patten, Scott M., Qiu, Huawei, Pan, Clark Qun, Edmunds, Tim

“…Gaucher disease is caused by mutations in the enzyme acid β-glucosidase (GCase), the most common of which is the substitution of serine for asparagine at…”
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Oxidation of Methionine Residues in Antithrombin by Van Patten, Scott M., Hanson, Eric, Bernasconi, Richard, Zhang, Kate, Manavalan, Partha, Cole, Edward S., McPherson, John M., Edmunds, Tim

“…Commercially available human plasma-derived preparations of the serine protease inhibitor antithrombin (AT) were shown to contain low levels of oxidation, and…”
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Impact of cysteine variants on the structure, activity, and stability of recombinant human [alpha]-galactosidase A by Qiu, Huawei, Honey, Denise M, Kingsbury, Jonathan S, Park, Anna, Boudanova, Ekaterina, Wei, Ronnie R, Pan, Clark Q, Edmunds, Tim

“…Recombinant human [alpha]-galactosidase A (rh[alpha]Gal) is a homodimeric glycoprotein deficient in Fabry disease, a lysosomal storage disorder. In this study,…”
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Structures of a pan‐specific antagonist antibody complexed to different isoforms of TGFβ reveal structural plasticity of antibody–antigen interactions by Moulin, Aaron, Mathieu, Magali, Lawrence, Catherine, Bigelow, Russell, Levine, Mark, Hamel, Christine, Marquette, Jean‐Piere, Parc, Josiane, Loux, Christophe, Ferrari, Paul, Capdevila, Cecile, Dumas, Jacques, Dumas, Bruno, Rak, Alexey, Bird, Julie, Qiu, Huawei, Pan, Clark Q., Edmunds, Tim, Wei, Ronnie R.

“…Various important biological pathways are modulated by TGFβ isoforms; as such they are potential targets for therapeutic intervention. Fresolimumab, also known…”
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