Protein Therapies

Bénichou B, Goyal S, Sung C, Norfleet AM, O'Brien F. 2009. A retrospective analysis of the potential impact of IgG antibodies to agalsidase beta on efficacy during enzyme replacement therapy for Fabry disease. Mol Genet Metab 96(1):4-12.

Dodge JC, Clarke J, Treleaven CM, Taksir TV, Griffiths DA, Yang W, Fidler JA, Passini MA, Karey KP, Schuchman EH, Cheng SH, Shihabuddin LS. 2009. Intracerebroventricular infusion of acid sphingomyelinase corrects CNS manifestations in a mouse model of Niemann-Pick A disease. Experimental Neuro 215(2):349-57.

Giugliani R, Rojas VM, Martins AM, Valadares ER, Clarke JTR, Goes JEC, Kakkis ED, Worden MA, Sidman M, Cox GF. 2009. A dose-optimization trial of laronidase (Aldurazyme®) in patients with mucopolysaccharidosis I. Molecular Genetics and Metabolism 96(1):13-19

Lubanda JC, Anijalg E, Bzdúch V, Thurberg BL, Bénichou B, Tylki-Szymanska A. 2009. Evaluation of a low dose, after a standard therapeutic dose, of agalsidase beta during enzyme replacement therapy in patients with Fabry disease. Genet Med 2009 Mar 4. [Epub ahead of print].

Bagley RG, Honma N, Weber W, Boutin P, Rouleau C, Shankara S, Kataoka S, Ishida I, Roberts BL, Teicher BA. 2008. Endosialin/TEM 1/CD248 is a pericyte marker of embryonic and tumor neovascularization. Microvasc Res 76(3):180-8.

Bagley RG, Rouleau C, St. Martin T, Boutin P, Weber W, Ruzek M, Honma N, Nacht M, Shankara S, Kataoka S, Ishida I, Roberts BL, Teicher BA.. 2008. Human endothelial precursor cells express tumor endothelial marker 1/endosialin/CD248. Mol Cancer Ther 7(8):2536-46.

Bodensteiner D, Scott CR, Sims KB, Shepherd GM, Cintron RD, Germain DP. 2008. Successful reinstitution of agalsidase beta therapy in Fabry disease patients with previous IgE-antibody or skin-test reactivity to the recombinant enzyme. Genetics in Medicine 10(5):353-8.

Chan CP. 2008. Biochemical and biophysical methods currently used for therapeutic protein development. Amer Pharma Rev 11(6):Nov 2008.

Chang M, Cooper JD, Sleat DE, Cheng SH, Dodge JC, Passini MA, Lobel P, Davidson BL. 2008. Intraventricular enzyme replacement improves disease phenotypes in a mouse model of Late Infantile Neuronal Ceroid Lipofuscinosis. Mol Ther 16(4):649-56.

Corzo D, Byrne B, Hwu W-L, Leslie N, Mandel H, Nicolino M. 2008. Alglucosidase Alfa (Myozyme®) in infants and children with rapidly progressive Pompe disease. Clinical Therapeutics 30(SUPPL. 1):S9-S10.

Dodge JC, Clarke J, Treleaven CM, Taksir TV, Griffiths DA, Yang W, Fidler JA, Passini MA, Karey KP, Schuchman EH, Cheng SH, Shihabuddin LS. 2008. Intracerebroventricular infusion of acid sphingomyelinase corrects CNS manifestations in a mouse model of Niemann-Pick A disease. Exp Neurol 2008 Nov 14. [Epub ahead of print].

Dodge JC, Haidet AM, Yang W, Passini MA, Hester M, Clarke J, Roskelley EM, Treleaven CM, Rizo L, Martin H, Kim SH, Kaspar R, Taksir TV, Griffiths DA, Cheng SH, Shihabuddin LS, Kaspar BK. 2008. Delivery of AAV-IGF-1 to the CNS extends survival in ALS mice through modification of aberrant glial cell activity. Mole Ther 16(6):1056-64.

Fattouh R, Midence G, Arias K, Johnson JR, Walker TD, Goncharova S, Souza KP, Gregory RC, Lonning S, Gauldie J, Jordana M. 2008. Transforming growth factor-β regulates house dust mite-induced allergic airway inflammation but not airway remodeling. Am J Respir Crit Care Med. 177(6):593-603.

Frantz S, Hu K, Adamek A, Wolf J, Sallam A, Maier SK, Lonning S, Ling H, Ertl G, Bauersachs J. 2008. Transforming growth factor beta inhibition increases mortality and left ventricular dilatation after myocardial infarction. Basic Res Cardiol 103(5):485-92.

Grütter C, Wilkinson T, Turner R, Podichetty S, Finch D, McCourt M, Lonning S, Jermutus L, Grütter MG. 2008. A cytokine-neutralizing antibody as a structural mimetic of 2 receptor interactions. Proc Natl Acad Sci U S A 105(51):20251-6.

Jiang Y, Howe MM. 2008. Regional mutagenesis of the gene encoding the phage Mu late gene activator C identifies two separate regions important for DNA binding. Nucleic Acids Res 36(20):6396-6405.

Kaplan J, Woodworth L, Smith K, Coco J, Vitsky A, McPherson J. 2008. Therapeutic benefit of treatment with anti-thymocyte globulin and latent TGF-beta1 in the MRL/lpr lupus mouse model. Lupus 17(9):822-31.

Kingsbury JS, Laue TM, Klimtchuk ES, Théberge R, Costello CE, Connors LH. 2008. The modulation of transthyretin tetramer stability by cysteine 10 adducts and the drug diflunisal: Direct analysis by fluorescence-detected analytical ultracentrifugation. J Biol Chem 283(18):11887-96.

McDowell R, Li JS, Benjamin Jr. DK, Morgan C, Becker A, Kishnani PS, Kanter RJ. 2008. Arrhythmias in patients receiving enzyme replacement therapy for infantile Pompe disease. Genet in Med 10(10):758-62.

McVie-Wylie AJ, Lee KL, Qiu H, Jin X, Do H, Gotschall R, Thurberg BL, Rogers C, Raben N, O'Callaghan M, Canfield W, Andrews L, McPherson JM, Mattaliano RJ. 2008. Biochemical and pharmacological characterization of different recombinant acid α-glucosidase preparations evaluated for the treatment of Pompe disease. Mol Genet Metab 94(4):448-55.

Moreno S, Ibraghimov-Beskrovnaya O, Bukanov NO. 2008. Serum and urinary biomarker signatures for rapid preclinical in vivo assessment of CDK inhibition as a therapeutic approach for PKD. Cell Cycle 7(12):1856-64.

Nam JS, Terabe M, Kang MJ, Chae H, Voong N, Yang YA, Laurence A, Michalowska A, Mamura M, Lonning S, Berzofsky JA, Wakefield LM. 2008. Transforming growth factor beta subverts the immune system into directly promoting tumor growth through interleukin-17. Cancer Res 68(10):3915-23.

Nam JS, Terabe M, Mamura M, Kang MJ, Chae H, Stuelten C, Kohn E, Tang B, Sabzevari H, Anver MR, Lawrence S, Danielpour D, Lonning S, Berzofsky JA, Wakefield LM. 2008. An anti-transforming growth factor beta antibody suppresses metastasis via cooperative effects on multiple cell compartments. Cancer Res 68(10):3835-43.

Perry K, Wong L, Liu V, Park I, Zhang Q, Rejen V, Huang X, Smith ND, Jovanovic B, Lonning S Teicher BA, Lee C. 2008. Treatment of transforming growth factor-beta-insensitive mouse renca tumor by transforming growth factor-beta elimination. Urology 72 (1):225-9.

Qi W, Zhang A, Patel D, Lee S, Harrington JL, Zhao L, Schaefer D, Good TA, Fernandez EJ. 2008. Simultaneous monitoring of peptide aggregate distributions, structure, and kinetics using amide hydrogen exchange: Application to Aβ(1-40) fibrillogenesis. Biotech Bioengin 100(6):1214-27.

Rouleau C, Curiel M, Weber W, Smale R, Kurtzberg L, Mascarello J, Berger C, Wallar G, Bagley R, Honma N, Hasegawa K, Ishida I, Kataoka S, Thurberg BL, Mehraein K, Horten B, Miller G, Teicher BA. 2008. Endosialin protein expression and therapeutic target potential in human solid tumors: sarcoma versus carcinoma. Clin Cancer Res 14(22):7223-36.

Rouleau C, Menon K, Boutin P, Guyre C, Yoshida H, Kataoka S, Perricone M, Shankara S, Frankel AE, Duesbery NS, Vande Woude G, Biemann H-P, Teicher BA. 2008. The systemic administration of lethal toxin achieves a growth delay of human melanoma and neuroblastoma xenografts: Assessment of receptor contribution. Intl J of Oncology 32(4):739-48.

Ruzek MC, Waire JS, Hopkins D, Lacorcia G, Sullivan J, Roberts BL, Richards SM, Nahill SR, Williams JM, Scaria A, Dzuris J, Shankara S, Garman RD. 2008. Characterization of in vitro antimurine thymocyte globulin-induced regulatory T cells that inhibit graft-versus-host disease in vivo. Blood 111:1726-1734.

Simon G, Parker M, Ramiya V, Wasserfall C, Huang Y, Bresson D, Schwartz RF, Campbell-Thompson M, Tenace L, Brusko, T, Xue S, Scaria A, Lukason M, Eisenbeis S, Williams J, Clare-Salzler M, Schatz D, Kaplan B, Von Herrath M, Womer K, Atkinson MA. 2008. Murine antithymocyte globulin therapy alters disease progression in NOD mice by a time-dependent induction of immunoregulation. Diabetes 57(2):405-14.

Sun L, Vukicevic S, Baliram R, Yang G, Sendak R, McPherson J, Zhu L-L, Iqbal J, Latif .R, Natrajan A, Arabi A, Yamoah K, Moonga BS, Gabet Y, Davies TF, Bab I, Abe E, Sampath K, Zaidi M. 2008. Intermittent recombinant TSH injections prevent ovariectomy-induced bone loss. Proc. of the Natl. Acad. of Sci. of the U.S.A. 105 (11):4289-94.

Wahome J, Zhou W, Kundu A. 2008. Impact of lot-to-lot variability of cation exchange chromatography resin on process performance. BioPharm International 21(5):48-56.

Weinreb N, Taylor J, Cox T, Yee J, Vom Dahl S. 2008. A benchmark analysis of the achievement of therapeutic goals for type 1 Gaucher disease patients treated with imiglucerase. Amer J Hematology 83(12):890-5.

Wilcox WR, Oliveira JP, Hopkin RJ, Ortiz A, Banikazemi M, Feldt-Rasmussen U, Sims K, Waldek S, Pastores GM, Lee P, Eng CM, Marodi L, Stanford KE, Breunig F, Wanner C, Warnock,DG, Lemay RM, Germain DP. 2008. Females with Fabry disease frequently have major organ involvement: Lessons from the Fabry Registry. Mol Genet Metab 93(2):112-28.

Yoshimizu M, Tajima Y, Matsuzawa F, Aikawa S-i, IIwamoto K, Kobayashi T, Edmunds T, Fujishima K, Tsuji D, Itoh K, Ikekita M, Kawashima I, Sugawara K, Ohyanagi N, Suzuki T, Togawa T, Ohno K, Sakuraba H. 2008. Binding parameters and thermodynamics of the interaction of imino sugars with a recombinant human acid α-glucosidase (alglucosidase alfa): Insight into the complex formation mechanism. Clinica Chimica Acta 391(1-2):68-73.

Zhou Q, Shankara S, Roy A, Qiu H, Estes S, McVie-Wylie A, Culm-Merdek K, Park A, Pan C, Edmunds T. 2008. Development of a simple and rapid method for producing nonfucosylated oligomannose containing antibodies with increased effector function. Biotechnol Bioeng 99:652-665.

Berndt TJ, Craig TA, McCormick DJ, Lanske B, Sitara D, Razzaque MS, Pragnell M, Bowe AE, O'Brien SP, Schiavi SC, Kumar R. 2007. Biological activity of FGF-23 fragments. Pflugers Archiv EU J Physiol 454:615-623.

Brown C, Selig WM, Ellis JL. 2007. Modulation of citric acid-induced cough following lipopolysaccharide-mediated neutrophilia in the guinea pig. Pulm Pharmacol Ther 20:90-97.

Case LE, Hanna R, Frush DP, Krishnamurthy V, DeArmey S, Mackey J, Boney A, Morgan C, Corzo D,

Bouchard S, Weber TJ, Chen YT, Kishnani PS. 2007. Fractures in children with Pompe disease: a potential long-term complication. Pediatric Radiol 37:437-445.

Charrow J, Dulisse B, Grabowski GA, Weinreb NJ. 2007. The effect of enzyme replacement therapy on bone crisis and bone pain in patients with type 1 Gaucher disease. Clin Genet 71(3):205-11.

Chavez CA, Bohnsack RN, Kudo M, Gotschall RR, Canfield WM, Dahms NM. 2007. Domain 5 of the cation-independent mannose 6-phosphate receptor preferentially binds phosphodiesters (mannose 6-phosphate N-acetylglucosamine ester) Biochemistry 46(44):12604-17

Demaria CT, Cairns V, Schwarz C, Zhang J, Guerin M, Zuena E, Estes S, Karey KP. 2007. Accelerated clone selection for recombinant CHO cells using a FACS-based high-throughput screen. Biotechnol Prog. based high-throughput screen. Biotechnol Prog 23:465-472.

Denton CP, Merkel PA, Furst DE, Khanna D, Emery P, Hsu VM, Silliman N, Streisand J, Powell J, Akesson A, Coppock J, Hoogen F, Herrick A, Mayes MD, Veale D, Haas J, Ledbetter S, Korn JH, Black CM, Seibold JR. 2007. Recombinant human anti-transforming growth factor beta1 antibody therapy in systemic sclerosis: a multicenter, randomized, placebo-controlled phase I/II trial of CAT-192. Arthritis Rheum 56:323-333.

Donovan JL, Schroeder WS, Tran MT, Foster K, Forrest A, Lee TB, Gandhi PJ. 2007. Assessment of eptifibatide dosing in renal impairment before and after in-service education provided by pharmacists. J Managed Care Pharm 13(7):598-606.

Edmunds T. 2007. Designing protein therapies: Lessons learned from Cerezyme for Gaucher Disease. Clin Ther 29 (SUPPL. C):S75-S76.

El Chaar M, Chen J, Seshan SV, Jha S, Richardson I, Ledbetter SR, Vaughan JED, Poppas DP, Felsen D. 2007. Effect of combination therapy with enalapril and the TGF-beta antagonist 1D11 in unilateral ureteral obstruction. Amer J of Physiology 292:F1291-1301.

Gupta S, Indelicato SR, Jethwa V, Kawabata T, Kelley M, Mire-Sluis AR, Richards SM, Rup B, Shores E, Swanson SJ, Wakshull E. 2007. Recommendations for the design, optimization, and qualification of cell-based assays used for the detection of neutralizing antibody responses elicited to biological therapeutics. J Immunol Methods 321:1-18.

Halbrooks PJ, Ding R, Wozney JM, Bain G. 2007. Role of RGM coreceptors in bone morphogenetic protein signaling. J Mol Signal 2:4.

Ikegami M, Scoville EA, Grant S, Korfhagen T, Brondyk W, Scheule RK, Whitsett JA. 2007. Surfactant protein-D and surfactant inhibit endotoxin-induced pulmonary inflammation. Chest 132:1447-1454.

Jongen SP, Gerwig GJ, Leeflang BR, Koles K, Mannesse ML, van Berkel PH, Pieper FR, Kroos MA, Reuser AJ, Zhou Q, Jin X, Zhang K, Edmunds T, Kamerling JP. 2007. N-glycans of recombinant human acid alpha-glucosidase expressed in the milk of transgenic rabbits. Glycobiology 17:600-619.

Kishnani PS, Corzo D, Nicolino M, Byrne B, Mandel H, Hwu WL, Leslie N, Levine J, Spencer C, McDonald M, Li J, Dumontier J, Halberthal M, Chien YH, Hopkin R, Vijayaraghavan S, Gruskin D, Bartholomew D, van der Ploeg A, Clancy JP, Parini R, Morin G, Beck M, De la Gastine GS, Jokic M, Thurberg B, Richards S, Bali D, Davison M, Worden MA, Chen YT, Wraith JE. 2007. Recombinant human acid [alpha]-glucosidase: major clinical benefits in infantile-onset Pompe disease. Neurology 68:88-89.

Nakanishi H, Sugiura T, Streisand JB, Lonning SM, Roberts JDJ. 2007. TGF-beta-neutralizing antibodies improve pulmonary alveologenesis and vasculogenesis in the injured newborn lung. Am J Physiol Lung Cell Mol Physiol 293:L151-161.

Palmer RE, Amartino HM, Niizawa G, Blanco M, Pomponio RJ, Chamoles NA. 2007. Pompe disease (glycogen storage disease type II) in Argentineans: Clinical manifestations and identification of 9 novel mutations. Neuromuscul Disord 17:16-22.

Prentice HL, Tonkin CJD, Caamano L, Sisk WP. 2007. High level expression of proteins using sequences from the ferritin heavy chain gene locus. J Biotechnology 128(1):50-60.

Reczek D, Schwake M, Schröder J, Hughes H, Blanz J, Jin X, Brondyk W, Van Patten S, Edmunds T, Saftig P. 2007. LIMP-2 is a receptor for lysosomal mannose-6-phosphate-independent targeting of beta-glucocerebrosidase. Cell 131(4):770-83.

Riske F, Smith M, Menon M, Goetschalck S, Goidsenhoven IV, Krul A, Pimpaneau V, Renaers I, Van Tichelt N, Van Houdt K, Hayes M, Lawrence C, Bigelow R, Schroeder J. 2007. A potential generic downstream process using Cibracon Blue resin at very high loading capacity produces a highly purified monoclonal antibody preparation from cell culture harvest. J of Chromatography B 848:108-115.

Starzyk K, Richards S, Yee J, Smith SE, Kingma W. 2007. The long-term international safety experience of imiglucerase therapy for Gaucher disease. Mol Genet Metab 90:157-163.

Swinnen K, Krul A, Van Goidsenhoven I, Van Tichelt N, Roosen A, Van Houdt K. 2007. Performance comparison of protein A affinity resins for the purification of monoclonal antibodies. J Chromatogr B Analyt Technol Biomed Life Sci 848:97-107.

Taksir TV, Griffiths D, Johnson J, Ryan S, Shihabuddin LS, Thurberg BL. 2007. Optimized preservation of CNS morphology for the identification of glycogen in the Pompe mouse model. J Histochem Cytochem 55:991-998.

Van Patten SM, Hughes H, Huff MR, Piepenhagen PA, Waire J, Qiu H, Ganesa C, Reczek D, Ward PV, Kutzko JP, Edmunds T. 2007. Effect of mannose chain length on targeting of glucocerebrosidase for enzyme replacement therapy of Gaucher disease. Glycobiology.

Velayudhan A, Menon MK. 2007. Modeling of purification operations in biotechnology: enabling process development, optimization, and scale-up. Biotechnol Prog 23:68-73.

Weinreb NJ, Barranger J, Packman S, Prakash-Cheng A, Rosenbloom B, Sims K, Angell J, Skrinar A, Pastores GM. 2007. Imiglucerase (Cerezyme) improves quality of life in patients with skeletal manifestations of Gaucher disease. Clin Genet 71:576-588.

Benigni A, Zoja C, Campana M, Corna D, Sangalli F, Rottoli D, Gagliardini E, Conti S, Ledbetter S, Remuzzi G. 2006. Beneficial effect of TGFbeta antagonism in treating diabetic nephropathy depends on when treatment is started. Nephron - Exper Nephrol 104:e158-168.

Berndt TJ, Bielesz B, Craig TA, Tebben PJ, Bacic D, Wagner CA, O'Brien S, Schiavi S, Biber J, Murer H, Kumar R. 2006. Secreted frizzled-related protein-4 reduces sodium-phosphate co-transporter abundance and activity in proximal tubule cells. Pflugers Arch 451:579-587.

Bukanov NO, Smith LA, Klinger KW, Ledbetter S, Ibraghimov-Beskrovnaya O. 2006. Long-lasting arrest of murine polycystic kidney disease with CDK inhibitor roscovitine. Nature 444:949-952.

Fukuda T, Ewan L, Bauer M, Mattaliano RJ, Zaal K, Ralston E, Plotz PH, Raben N. 2006. Dysfunction of endocytic and autophagic pathways in a lysosomal storage disease. Ann Neurol 59:700-708.

Ikegami M, Carter K, Bishop K, Yadav A, Masterjohn E, Brondyk W, Scheule RK, Whitsett JA. 2006. Intratracheal recombinant surfactant protein d prevents endotoxin shock in the newborn preterm lamb. Am J Respir Crit Care Med 173:1342-1347.

Jobling MF, Mott JD, Finnegan MT, Jurukovski V, Erickson AC, Walian PJ, Taylor SE, Ledbetter S, Lawrence CM, Rifkin DB, Barcellos-Hoff MH. 2006. Isoform-specific activation of latent transforming growth factor beta (LTGF-beta) by reactive oxygen species. Radiation Research 166:839-848.

Morin R, Kaplan D, Perez-Ramirez B. 2006. Bone morphogenetic protein-2 binds as multilayers to a collagen delivery matrix: An equilibrium thermodynamic analysis. Biomacromole 7:131-138.

Nam JS, Suchar AM, Kang MJ, Stuelten CH, Tang B, Michalowska AM, Fisher LW, Fedarko NS, Jain A, Pinkas J, Lonning S, Wakefield LM. 2006. Bone sialoprotein mediates the tumor cell-targeted prometastatic activity of transforming growth factor beta in a mouse model of breast cancer. Cancer Res 66:6327-6335.

Pande S, Ritter CS, Rothstein M, Wiesen K, Vassiliadis J, Kumar R, Schiavi SC, Slatapolsky E, Brown AJ. 2006. FGF-23 and sFRP-4 in Chronic Kidney Disease and post-renal transplantation. Nephron Physiol 104:23-32.

Rapoza ML, Fu D, Sendak RA. 2006. Development of an in vitro potency assay for therapeutic TGFbeta antagonists: the A549 cell bioassay. J Immunol Methods 316:18-26.

Riske F, Smith M, Menon M, Goetschalck S, Goidsenhoven IV, Krul A, Pimpaneau V, Renaers I, Van Tichelt N, Van Houdt K, Hayes M, Lawrence C, Bigelow R, Schroeder J. 2006. A potential generic downstream process using Cibracon Blue resin at very high loading capacity produces a highly purified monoclonal antibody preparation from cell culture harvest. J Chromatogr B Analyt Technol Biomed Life Sci.

Rouleau C, Roy A, St Martin T, Dufault MR, Boutin P, Liu D, Zhang M, Puorro-Radzwill K, Rulli L, Reczek D, Bagley R, Byrne A, Weber W, Roberts B, Klinger K, Brondyk W, Nacht M, Madden S, Burrier R, Shankara S, Teicher BA. 2006. Protein tyrosine phosphatase PRL-3 in malignant cells and endothelial cells: Expression and function. Mol Cancer Ther 5:219-229.

Sawkar AR, Schmitz M, Zimmer KP, Reczek D, Edmunds T, Balch WE, Kelly JW. 2006. Chemical chaperones and permissive temperatures alter localization of Gaucher disease associated glucocerebrosidase variants. ACS Chem Biol 1:235-251.

Schiavi SC. 2006. Bone talk. Nat Genet 38:1230-1231.

Smith LA, Bukanov NO, Husson H, Russo RJ, Barry TC, Taylor AL, Beier DR, Ibraghimov-Beskrovnaya O. 2006. Development of polycystic kidney disease in juvenile cystic kidney mice: insights into pathogenesis, ciliary abnormalities, and common features with human disease. J Am Soc Nephrol 17:2821-2831.

Syal A, Schiavi S, Chakravarty S, Dwarakanath V, Quigley R, Baum M. 2006. Fibroblast growth factor-23 increases mouse PGE2 production in vivo and in vitro. Am J Physiol Renal Physiol 290:F450-455.

Berndt TJ, Bielesz B, Craig TA, Tebben PJ, Bacic D, Wagner CA, O'Brien S, Schiavi S, Biber J, Murer H, Kumar R. 2005. Secreted frizzled-related protein-4 reduces sodium-phosphate co-transporter abundance and activity in proximal tubule cells. Pflugers Arch.

Thurberg BL, Lynch Maloney C, Vaccaro C, Afonso K, Tsai AC, Bossen E, Kishnani PS, O'Callaghan M. 2006. Characterization of pre- and post-treatment pathology after enzyme replacement therapy for Pompe disease. Lab Invest 86:1208-1220.

Zhang H, Kallwass H, Young SP, Carr C, Dai J, Kishnani PS, Millington DS, Keutzer J, Chen YT, Bali D. 2006. Comparison of maltose and acarbose as inhibitors of maltase-glucoamylase activity in assaying acid alpha-glucosidase activity in dried blood spots for the diagnosis of infantile Pompe disease. Genet Med 8:302-306.

An Y, Young SP, Kishnani PS, Millington DS, Amalfitano A, Corzo D, Chen YT. 2005. Glucose tetrasaccharide as a biomarker for monitoring the therapeutic response to enzyme replacement therapy for Pompe disease. Mol Genet Metab 85:247-254.

Banovic T, MacDonald KP, Morris ES, Rowe V, Kuns R, Don A, Kelly J, Ledbetter S, Clouston AD, Hill GR. 2005. TGF-beta in allogeneic stem cell transplantation: friend or foe? Blood 106:2206-2214.

De Vry CG, Valdez M, Lazarov M, Muhr E, Buelow R, Fong T, Iyer S. 2005 Topical application of a novel immunomodulatory peptide, RDP58, reduces skin inflammation in the phorbol ester-induced dermatitis model. J Invest Dermatol 125(3):473-481.

Du H, Levine M, Ganesa C, Witte DP, Cole ES, Grabowski GA. 2005. The role of mannosylated enzyme and the mannose receptor in enzyme replacement therapy. Am J Hum Genet 77:1061-1074.

Lavoie P, Robitaille G, Agharazii M, Ledbetter S, Lebel M, Lariviere R. 2005. Neutralization of transforming growth factor-beta attenuates hypertension and prevents renal injury in uremic rats. J Hypertens 23:1895-1903.

Lu L, Saulis AS, Liu WR, Roy NK, Chao JD, Ledbetter S, Mustoe TA. 2005. The temporal effects of anti-TGF-beta1, 2, and 3 monoclonal antibody on wound healing and hypertrophic scar formation. J Am Coll Surg 201:391-397.

Perez-Ramirez B, Steckert JJ. 2005. Probing reversible self-association of therapeutic proteins by sedimentation velocity in the analytical ultracentrifuge. Methods Mol Biol 308:301-318.

Zhou Q, Kyazike J, Echelard Y, Meade HM, Higgins E, Cole ES, Edmunds T. 2005. Effect of genetic background on glycosylation heterogeneity in human antithrombin produced in the mammary gland of transgenic goats. J Biotechnol 117:57-72.

Borovecki F, Jelic M, Grgurevic L, Sampath KT, Bosukonda D, Vukicevic S. 2004. Bone morphogenetic protein-7 from serum of pregnant mice is available to the fetus through placental transfer during early stages of development. Nephron Exp Nephrol 97:e26-32.

Magner JA. 2004. Emil Fischer (1852-1919). The stereochemical nature of sugars. Endocrinologist 14:239-244.

Nelson BC, Roddy T, Araghi S, Wilkens D, Thomas JJ, Zhang K, Sung CC, Richards SM. 2004. Globotriaosylceramide isoform profiles in human plasma by liquid chromatography-tandem mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci 805:127-134.

Schiavi SC, Kumar R. 2004. The phosphatonin pathway: new insights in phosphate homeostasis. Kidney Int 65:1-14.

Stuart ES, Morshed F, Sremac M, Dassarma S. 2004. Cassette-based presentation of SIV epitopes with recombinant gas vesicles from halophilic archaea. J Biotechnol 114:225-237.

Xu Y, Kolber-Simonds D, Hope JA, Bazin H, Latinne D, Monroy R, White-Scharf ME, Schuurman HJ. 2004. The anti-CD2 monoclonal antibody BTI-322 generates unresponsiveness by activation-associated T cell depletion. Clin & Exper Immunol 138:476-483.

Zhou Q, Park SH, Boucher S, Higgins E, Lee K, Edmunds T. 2004. N-linked oligosaccharide analysis of glycoprotein bands from isoelectric focusing gels. Anal Biochem 335:10-16.

Benigni A, Zoja C, Corna D, Zatelli C, Conti S, Campana M, Gagliardini E, Rottoli D, Zanchi C, Abbate M, Ledbetter S, Remuzzi G. 2003. Add-on anti-TGF-beta antibody to ACE inhibitor arrests progressive diabetic nephropathy in the rat. J Am Soc Nephrol 14:1816-1824.

Ganesa C, Granda BW, Mattaliano RJ. 2003. Sialylation levels influence oligosaccharide quantitation: analyzing response variability using high-pH anion-exchange chromatography and pulsed amperometric detection. BioPharm Intl 16:44, 46-48, 50, 52.

Lanciotti J, Song A, Doukas J, Sosnowski B, Pierce G, Gregory R, Wadsworth S, O'Riordan C. 2003. Targeting adenoviral vectors using heterofunctional polyethylene glycol FGF2 conjugates. Mole Ther 8:99-107.

Lee K, Jin X, Zhang K, Copertino L, Andrews L, Baker-Malcolm J, Geagan L, Qiu H, Seiger K, Barngrover D, McPherson J, Edmunds T. 2003. A biochemical and pharmacological comparison of enzyme replacement therapies for the glycolipid storage disorder Fabry disease. Glycobiology 13:305-313.

Ling H, Li X, Jha S, Wang W, Karetskaya L, Pratt B, Ledbetter S. 2003. Therapeutic role of TGF-beta-neutralizing antibody in mouse cyclosporin A nephropathy: morphologic improvement associated with functional preservation. J Am Soc Nephrol 14:377-388.

Maric I, Poljak L, Zoricic S, Bobinac D, Bosukonda D, Sampath KT, Vukicevic S. 2003. Bone morphogenetic protein-7 reduces the severity of colon tissue damage and accelerates the healing of inflammatory bowel disease in rats. J Cell Physiol 196:258-264.

Sampath TK, Reddi AH. 2003. Bone morphogenetic protein (BMP) implants as bone graft substitutes. Promises and challenges. In: Laurencin CT, editor. Bone Graft Subs [Proc Wksp, Orlando, FL 2000]: ASTM International and The American Academy of Orthopedic Surgeons. p 194-213.

Sendak RA, Ganesa C, Lee KL, Harrahy JJ, Theberge R, Morgan CJ, Cole ES, Kohn LD, Mattaliano RJ. 2003. The effect of posttranslational modifications on the in vitro activity of recombinant human thyroid-stimulating hormone. Thyroid 13:1091-1101.

Streets AJ, Newby LJ, O'Hare MJ, Bukanov NO, Ibraghimov-Beskrovnaya O, Ong AC. 2003. Functional analysis of PKD1 transgenic lines reveals a direct role for polycystin-1 in mediating cell-cell adhesion. J Am Soc Nephrol 14:1804-1815.

Sumner DR, Turner TM, Cohen M, Losavio P, Urban RM, Nichols EH, McPherson JM. 2003. Aging does not lessen the effectiveness of TGFbeta2-enhanced bone regeneration. J Bone Miner Res 18:730-736.

Barngrover D. 2002. Fabrazyme--recombinant protein treatment for Fabry's disease. J Biotech 95:280-282.

Dahly AJ, Hoagland KM, Flasch AK, Jha S, Ledbetter SR, Roman RJ. 2002. Antihypertensive effects of chronic anti-TGF-beta antibody therapy in Dahl S rats. Am J Physiol Regul Integr Comp Physiol 283:R757-767.

Sendak RA, Wang F, Geagan LB, Armstrong LA, Thyne CD, Cole ES, Mattaliano RJ. 2002. Comparison of two in vitro methods for the measurement of recombinant human TSH bioactivity. Biologicals 30:245-254.

Zhou Q, Kyazike J, Edmunds T, Higgins E. 2002. Mannose 6-phosphate quantitation in glycoproteins using high-pH anion-exchange chromatography with pulsed amperometric detection. Anal Biochem 306:163-170.