| 1 |
Production of goats by somatic cell nuclear transfer
Alexander B. Baguisi, Esmail Behboodi, David T. Melican et al. · Nature Biotechnology
|
May 1999 |
1,005 |
| 2 |
LIMP-2 Is a Receptor for Lysosomal Mannose-6-Phosphate-Independent Targeting of β-Glucocerebrosidase
David Reczek, Michael Schwake, J Oltmann Schröder et al. · Cell
Open access |
Nov 2007 |
538 |
| 3 |
Transforming Growth Factor-β Production and Myeloid Cells Are an Effector Mechanism through Which CD1d-restricted T Cells Block Cytotoxic T Lymphocyte–mediated Tumor Immunosurveillance
Masaki Terabe, So Matsui, Jong-Myun Park et al. · The Journal of Experimental Medicine
Open access |
Dec 2003 |
538 |
| 4 |
Globotriaosylceramide accumulation in the Fabry kidney is cleared from multiple cell types after enzyme replacement therapy
Beth L. Thurberg, Helmut G. Rennke, Robert B. Colvin et al. · Kidney International
Open access |
Dec 2002 |
335 |
| 5 |
CNS expression of glucocerebrosidase corrects α-synuclein pathology and memory in a mouse model of Gaucher-related synucleinopathy
S. Pablo Sardi, Jennifer Leigh Clarke, Cathrine Kinnecom et al. · Proceedings of the National Academy of Sciences
Open access |
5 Jul 2011 |
324 |
| 6 |
Characterization of Fabry Disease in 352 Pediatric Patients in the Fabry Registry
Robert James Hopkin, John J. Bissler, Maryam Banikazemi et al. · Pediatric Research
|
9 Oct 2008 |
306 |
| 7 |
CNS-targeted gene therapy improves survival and motor function in a mouse model of spinal muscular atrophy
Marco A. Passini, Jie Bu, Eric M. Roskelley et al. · Journal of Clinical Investigation
Open access |
16 Mar 2010 |
298 |
| 8 |
Discovery and development of clofarabine: a nucleoside analogue for treating cancer
Peter L. Bonate, Larry E. Arthaud, William R. Jr. Cantrell et al. · Nature Reviews Drug Discovery
Review |
Oct 2006 |
264 |
| 9 |
Exploring the Specificity of Nanozymes
Huizhen Fan, Ruofei Zhang, Kelong Fan et al. · ACS Nano
|
12 Jan 2024 |
261 |
| 10 |
The phosphatonin pathway: New insights in phosphate homeostasis
Susan C. Schiavi, Rajiv Kumar · Kidney International
Open access |
16 Dec 2003 |
253 |
| 11 |
Reduced Inflammatory Response to Plasmid DNA Vectors by Elimination and Inhibition of Immunostimulatory CpG Motifs
Nelson S. Yew, Hongmei Zhao, I‐Huan Wu et al. · Molecular Therapy
Open access |
Mar 2000 |
224 |
| 12 |
CpG-Depleted Plasmid DNA Vectors with Enhanced Safety and Long-Term Gene Expression in Vivo
Nelson S. Yew, Hongmei Zhao, Malgorzata Przybylska et al. · Molecular Therapy
Open access |
Jun 2002 |
223 |
| 13 |
Tumors Associated With Oncogenic Osteomalacia Express Genes Important in Bone and Mineral Metabolism
Suzanne M. Jan de Beur, Richard B. Finnegan, John Vassiliadis et al. · Journal of Bone and Mineral Research
|
Jun 2002 |
217 |
| 14 |
Induction of Antitumor Immunity with Dendritic Cells Transduced with Adenovirus Vector-Encoding Endogenous Tumor-Associated Antigens
Johanne M Kaplan, Queendy Yu, Susan Piraino et al. · The Journal of Immunology
|
Jul 1999 |
206 |
| 15 |
Collagen matrices attenuate the collagen-synthetic response of cultured fibroblasts to TGF-β
Richard A.F. Clark, Larry D. Nielsen, Marshall P. Welch et al. · Journal of Cell Science
|
Mar 1995 |
203 |
| 16 |
Development of a simple and rapid method for producing non‐fucosylated oligomannose containing antibodies with increased effector function
Qun Zhou, Srinivas Shankara, Andre J Roy et al. · Biotechnology and Bioengineering
|
6 Aug 2007 |
189 |
| 17 |
The Temporal Effects of Anti-TGF-β1, 2, and 3 Monoclonal Antibody on Wound Healing and Hypertrophic Scar Formation
Leonard Lu, Alexandrina S. Saulis, W. Robert Liu et al. · Journal of the American College of Surgeons
|
12 Jul 2005 |
177 |
| 18 |
Glycoengineered Acid α-Glucosidase With Improved Efficacy at Correcting the Metabolic Aberrations and Motor Function Deficits in a Mouse Model of Pompe Disease
Yunxiang Zhu, Ji-Lei Jiang, Nathan Gumlaw et al. · Molecular Therapy
Open access |
10 Mar 2009 |
176 |
| 19 |
Intracranial Delivery of CLN2 Reduces Brain Pathology in a Mouse Model of Classical Late Infantile Neuronal Ceroid Lipofuscinosis
Marco A. Passini, James C. Dodge, Jie Bu et al. · Journal of Neuroscience
Open access |
Feb 2006 |
171 |
| 20 |
Transforming Growth Factor-β and the Immune Response to Malignant Disease
Beverly A. Teicher · Clinical Cancer Research
|
Nov 2007 |
158 |
| 21 |
Cardiac Microvascular Pathology in Fabry Disease
Beth L. Thurberg, John T. Fallon, Richard N. Mitchell et al. · Circulation
Open access |
4 May 2009 |
155 |
| 22 |
Competing Risks for Death and Cardiac Transplantation in Children With Dilated Cardiomyopathy
Jorge A. Alvarez, Endel John Orav, James D. Wilkinson et al. · Circulation
Open access |
26 Jul 2011 |
153 |
| 23 |
Cysteine proteases as targets for metal-based drugs
Simon Paul Fricker · Metallomics
|
2010 |
143 |
| 24 |
Improvement of bone disease by imiglucerase (Cerezyme) therapy in patients with skeletal manifestations of type 1 Gaucher disease: results of a 48‐month longitudinal cohort study
KB Sims, GM Pastores, NJ Weinreb et al. · Clinical Genetics
Open access |
7 Apr 2008 |
135 |
| 25 |
High and Sustained Transgene Expression in Vivo from Plasmid Vectors Containing a Hybrid Ubiquitin Promoter
Nelson S. Yew, Malgorzata Przybylska, Robin J. Ziegler et al. · Molecular Therapy
Open access |
Jul 2001 |
135 |
| 26 |
Modification of an Adenoviral Vector with Biologically Selected Peptides: A Novel Strategy for Gene Delivery to Cells of Choice
Helen Romanczuk, Chad E. Galer, Joseph Zabner et al. · Human Gene Therapy
|
10 Nov 1999 |
135 |
| 27 |
The long-term international safety experience of imiglucerase therapy for Gaucher disease
Kathryn A. Starzyk, Susan M. Richards, John D. Yee et al. · Molecular Genetics and Metabolism
|
2 Nov 2006 |
123 |
| 28 |
Healing of chondral and osteochondral defects in a canine model: the role of cultured chondrocytes in regeneration of articular cartilage
Sonya Shortkroff, Leesa M. Barone, H.‐P. Hsu et al. · Biomaterials
|
1996 |
122 |
| 29 |
Prenatal diagnosis of 45,X and 45,X mosaicism: the need for thorough cytogenetic and clinical evaluations
Bing Huang, Maya Thangavelu, Sucheta Bhatt et al. · Prenatal Diagnosis
|
Feb 2002 |
120 |
| 30 |
Mechanism of cytochrome P450-3A inhibition by ketoconazole
David J. Greenblatt, Yanli Zhao, Karthik Venkatakrishnan et al. · Journal of Pharmacy and Pharmacology
Open access |
14 Jan 2011 |
120 |
| 31 |
A modified model of graft‐versus‐host–induced systemic sclerosis (scleroderma) exhibits all major aspects of the human disease
Melanie C. Ruzek, Sharda S. Jha, Steve Ledbetter et al. · Arthritis & Rheumatism
|
Apr 2004 |
120 |
| 32 |
Glucose tetrasaccharide as a biomarker for monitoring the therapeutic response to enzyme replacement therapy for Pompe disease
Yan An, Sarah P. Young, Priya Sunil Kishnani et al. · Molecular Genetics and Metabolism
|
11 May 2005 |
112 |
| 33 |
Reduced Thrombus Formation by Hyaluronic Acid Coating of Endovascular Devices
Stefan Verheye, Christos P. Markou, Mahomed Y. Salame et al. · Arteriosclerosis Thrombosis and Vascular Biology
Open access |
Apr 2000 |
112 |
| 34 |
Recombinant Human Thyroid Stimulating Hormone: Development of a Biotechnology Product for Detection of Metastatic Lesions of Thyroid Carcinoma
Edward S. Cole, Karen S. Lee, Kevin Lauziere et al. · Nature Biotechnology
|
Sep 1993 |
108 |
| 35 |
A retrospective analysis of the potential impact of IgG antibodies to agalsidase β on efficacy during enzyme replacement therapy for Fabry disease
Bernard Bénichou, Sunita Goyal, Crystal Sung et al. · Molecular Genetics and Metabolism
|
4 Dec 2008 |
106 |
| 36 |
Activation of the Unfolded Protein Response by ΔF508 CFTR
Rafał Bartoszewski, András Rab, Asta Jurkuvenaite et al. · American Journal of Respiratory Cell and Molecular Biology
Open access |
5 May 2008 |
105 |
| 37 |
Dose-response relationships for enzyme replacement therapy with imiglucerase/alglucerase in patients with Gaucher disease type 1
Gregory Alex Grabowski, Katherine A. Kacena, J. Alexander Cole et al. · Genetics in Medicine
Open access |
Feb 2009 |
102 |
| 38 |
Genomic analysis of partial 21q monosomies with variable phenotypes
Elisha D. O. Roberson, Elizabeth Wohler, Julie Elizabeth Hoover-Fong et al. · European Journal of Human Genetics
Open access |
8 Sep 2010 |
100 |
| 39 |
Long-term monitoring of patients with infantile-onset Pompe disease on enzyme replacement therapy using a urinary glucose tetrasaccharide biomarker
Sarah P. Young, Haoyue Zhang, Deyanira Corzo et al. · Genetics in Medicine
Open access |
Jul 2009 |
96 |
| 40 |
Electron Paramagnetic Resonance Imaging of Rat Heart with Nitroxide and Polynitroxyl-Albumin
Periannan Kuppusamy, Penghai Wang, Jay Louis Zweier et al. · Biochemistry
|
1996 |
93 |
| 41 |
Safety and efficacy assessment of plerixafor in patients with multiple myeloma proven or predicted to be poor mobilizers, including assessment of tumor cell mobilization
Guido J. K. Tricot, Michele Cottler‐Fox, Gary C Calandra · Bone Marrow Transplantation
|
22 Jun 2009 |
88 |
| 42 |
Cardiovascular manifestations of Fabry disease: relationships between left ventricular hypertrophy, disease severity, and -galactosidase A activity
Justina C. Wu, Carolyn Yung Ho, Hicham Skali et al. · European Heart Journal
Open access |
7 Jan 2010 |
86 |
| 43 |
A phase 2a, randomized, double‐blind 28‐day study of TZP‐102 a ghrelin receptor agonist for diabetic gastroparesis
Niels Ejskjær, John M. Wo, Tuba Esfandyari et al. · Neurogastroenterology & Motility
|
23 Dec 2012 |
86 |
| 44 |
Agalsidase beta treatment is associated with improved quality of life in patients with Fabry disease: Findings from the Fabry Registry
Torquil Watt, Alessandro P. Burlina, Chiara Cazzorla et al. · Genetics in Medicine
Open access |
29 Sep 2010 |
85 |
| 45 |
Immunomodulatory Activity of Mesenchymal Stem Cells
Johanne M Kaplan, Michele E. Youd, Tracey A. Lodie · Current Stem Cell Research & Therapy
|
Dec 2011 |
84 |
| 46 |
Impact of Shared Decision Making on Disease-Modifying Drug Adherence in Multiple Sclerosis
Aliza Bitton Ben‐Zacharia, Meagan Adamson, Allison N Boyd et al. · International Journal of MS Care
Open access |
Nov 2018 |
83 |
| 47 |
CFTR mutation distribution among U.S. Hispanic and African American individuals: Evaluation in cystic fibrosis patient and carrier screening populations
Elaine A. Sugarman, Elizabeth M. Rohlfs, Lawrence M. Silverman et al. · Genetics in Medicine
Open access |
Sep 2004 |
83 |
| 48 |
The use of dried blood spot samples in the diagnosis of lysosomal storage disorders — Current status and perspectives
Arnold J.J. Reuser, Frans W. Verheijen, Deeksha Sarihyan Bali et al. · Molecular Genetics and Metabolism
|
25 Jul 2011 |
83 |
| 49 |
Evaluation of Sepramesh Biosurgical Composite in a Rabbit Hernia Repair Model
Keith E. Greenawalt, Timothy J. Butler, Eric A. Rowe et al. · Journal of Surgical Research
|
Dec 2000 |
82 |
| 50 |
Human mesenchymal stem cells from bone marrow express tumor endothelial and stromal markers
Bagley · International Journal of Oncology
Open access |
9 Feb 2009 |
82 |