Published in Pediatr Res on March 01, 1998
Biochemical and pharmacological characterization of different recombinant acid alpha-glucosidase preparations evaluated for the treatment of Pompe disease. Mol Genet Metab (2008) 1.13
Glycosylation-independent lysosomal targeting of acid α-glucosidase enhances muscle glycogen clearance in pompe mice. J Biol Chem (2012) 1.04
Gene therapy for lysosomal storage diseases (LSDs) in large animal models. ILAR J (2009) 0.94
Comparative impact of AAV and enzyme replacement therapy on respiratory and cardiac function in adult Pompe mice. Mol Ther Methods Clin Dev (2015) 0.83
A review of treatment of Pompe disease in infants. Biologics (2007) 0.75
Recombinant human acid [alpha]-glucosidase: major clinical benefits in infantile-onset Pompe disease. Neurology (2006) 5.67
Epilepsy and exacerbation of brain injury in mice lacking the glutamate transporter GLT-1. Science (1997) 5.13
A survey of the humoral immune response of cancer patients to a panel of human tumor antigens. J Exp Med (1998) 5.06
Mechanisms of epithelial cell-cell adhesion and cell compaction revealed by high-resolution tracking of E-cadherin-green fluorescent protein. J Cell Biol (1998) 5.03
Simultaneous humoral and cellular immune response against cancer-testis antigen NY-ESO-1: definition of human histocompatibility leukocyte antigen (HLA)-A2-binding peptide epitopes. J Exp Med (1998) 4.65
Mutations in the dystrophin-associated protein gamma-sarcoglycan in chromosome 13 muscular dystrophy. Science (1995) 3.66
Recombinant human acid alpha-glucosidase enzyme therapy for infantile glycogen storage disease type II: results of a phase I/II clinical trial. Genet Med (2001) 3.60
Glycogen storage disease in adults. Ann Intern Med (1994) 3.58
Dead bird surveillance as an early warning system for West Nile virus. Emerg Infect Dis (2001) 3.32
New paths in human cancer serology. J Exp Med (1998) 3.12
Intragenic deletion in the gene encoding ubiquitin carboxy-terminal hydrolase in gad mice. Nat Genet (1999) 3.07
Induction of primary NY-ESO-1 immunity: CD8+ T lymphocyte and antibody responses in peptide-vaccinated patients with NY-ESO-1+ cancers. Proc Natl Acad Sci U S A (2000) 3.00
Chinese hamster ovary cell-derived recombinant human acid alpha-glucosidase in infantile-onset Pompe disease. J Pediatr (2006) 2.97
Immunohistochemical analysis of NY-ESO-1 antigen expression in normal and malignant human tissues. Int J Cancer (2001) 2.95
Monitoring CD8 T cell responses to NY-ESO-1: correlation of humoral and cellular immune responses. Proc Natl Acad Sci U S A (2000) 2.79
Predictors of readmission among elderly survivors of admission with heart failure. Am Heart J (2000) 2.71
Factors affecting synthesis of ascorbic acid in cress seedlings. 2. Ascorbic acid synthesis in relation to sugar formation. Biochem J (1949) 2.46
Evaluation of liver tumors using fluorine-18-fluorodeoxyglucose PET: characterization of tumor and assessment of effect of treatment. J Nucl Med (1992) 2.30
Humoral immune responses of cancer patients against "Cancer-Testis" antigen NY-ESO-1: correlation with clinical events. Int J Cancer (1999) 2.26
Altered cochlear fibrocytes in a mouse model of DFN3 nonsyndromic deafness. Science (1999) 2.25
Microstructural fingerprints of phase transitions in shock-loaded iron. Sci Rep (2013) 2.17
Dead crow densities and human cases of West Nile virus, New York State, 2000. Emerg Infect Dis (2001) 2.17
Fractal analysis of cerebral blood flow distribution in Alzheimer's disease. J Nucl Med (2001) 2.08
Response of capillary cell death to aminoguanidine predicts the development of retinopathy: comparison of diabetes and galactosemia. Invest Ophthalmol Vis Sci (2000) 2.06
Reliability of reported sunburn history in a case-control study of cutaneous malignant melanoma. Am J Epidemiol (1995) 2.05
Cloning of a brain protein identified by autoantibodies from a patient with paraneoplastic cerebellar degeneration. Proc Natl Acad Sci U S A (1987) 2.05
Serological identification of embryonic neural proteins as highly immunogenic tumor antigens in small cell lung cancer. Proc Natl Acad Sci U S A (2000) 2.02
A putative Ca2+-binding protein: structure of the light subunit of porcine calpain elucidated by molecular cloning and protein sequence analysis. Proc Natl Acad Sci U S A (1985) 1.99
Risk factors for cardiac and pyloric stenosis after endoscopic submucosal dissection, and efficacy of endoscopic balloon dilation treatment. Endoscopy (2009) 1.98
Genetic analysis of enterovirus 71 isolated from fatal and non-fatal cases of hand, foot and mouth disease during an epidemic in Taiwan, 1998. Virus Res (2000) 1.95
Antigens recognized by autologous antibody in patients with renal-cell carcinoma. Int J Cancer (1999) 1.91
Quantification of picogram levels of specific DNA immobilized in microtiter wells. FEBS Lett (1985) 1.90
Cornstarch therapy in type I glycogen-storage disease. N Engl J Med (1984) 1.88
Evasion of immune responses to introduced human acid alpha-glucosidase by liver-restricted expression in glycogen storage disease type II. Mol Ther (2005) 1.87
A mouse mutant p53 product recognized by CD4+ and CD8+ T cells. Proc Natl Acad Sci U S A (1994) 1.86
Analysis of the p53 gene and its expression in human glioblastoma cells. Cancer Res (1994) 1.84
Predicting one-year mortality among elderly survivors of hospitalization for an acute myocardial infarction: results from the Cooperative Cardiovascular Project. J Am Coll Cardiol (2001) 1.83
Characterization of human colon cancer antigens recognized by autologous antibodies. Int J Cancer (1998) 1.82
Nomenclature of the GLUT/SLC2A family of sugar/polyol transport facilitators. Am J Physiol Endocrinol Metab (2002) 1.82
Correlates and impact on outcomes of worsening renal function in patients > or =65 years of age with heart failure. Am J Cardiol (2000) 1.81
Identification of NY-ESO-1 peptide analogues capable of improved stimulation of tumor-reactive CTL. J Immunol (2000) 1.76
Frequent hypermethylation of CpG islands and loss of expression of the 14-3-3 sigma gene in human hepatocellular carcinoma. Oncogene (2000) 1.76
Influence of interleukin 12 on p53 peptide vaccination against established Meth A sarcoma. Proc Natl Acad Sci U S A (1995) 1.75
Interaction of folliculin (Birt-Hogg-Dubé gene product) with a novel Fnip1-like (FnipL/Fnip2) protein. Oncogene (2008) 1.74
A prognostic rule for elderly patients admitted with community-acquired pneumonia. Am J Med (1999) 1.74
Monophasic and biphasic synovial sarcomas abundantly express cancer/testis antigen NY-ESO-1 but not MAGE-A1 or CT7. Int J Cancer (2001) 1.71
Comparative specificity and kinetic studies on porcine calpain I and calpain II with naturally occurring peptides and synthetic fluorogenic substrates. J Biol Chem (1984) 1.70
Comparing AMI mortality among hospitals in patients 65 years of age and older: evaluating methods of risk adjustment. Circulation (1999) 1.69
Humoral immunity to human breast cancer: antigen definition and quantitative analysis of mRNA expression. Cancer Immun (2001) 1.69
The Colletotrichum lagenarium MAP kinase gene CMK1 regulates diverse aspects of fungal pathogenesis. Mol Plant Microbe Interact (2000) 1.69
Identification of NY-ESO-1 epitopes presented by human histocompatibility antigen (HLA)-DRB4*0101-0103 and recognized by CD4(+) T lymphocytes of patients with NY-ESO-1-expressing melanoma. J Exp Med (2000) 1.67
Differentiation and transforming growth factor-beta receptor down-regulation by collagen-alpha2beta1 integrin interaction is mediated by focal adhesion kinase and its downstream signals in murine osteoblastic cells. J Biol Chem (1997) 1.66
Early termination of heterogeneous nuclear RNA transcripts in mammalian cells: accentuation by 5,6-dichloro 1-beta-D-ribofuranosylbenzimidazole. Proc Natl Acad Sci U S A (1979) 1.66
Rapid diagnosis of MCAD deficiency: quantitative analysis of octanoylcarnitine and other acylcarnitines in newborn blood spots by tandem mass spectrometry. Clin Chem (1997) 1.65
Quality of care for patients hospitalized with heart failure at academic medical centers. Am Heart J (1999) 1.65
Characteristics of t(8;21) acute myeloid leukemia (AML) with additional chromosomal abnormality: concomitant trisomy 4 may constitute a distinctive subtype of t(8;21) AML. Leukemia (2003) 1.63
Human glycogen debranching enzyme gene (AGL): complete structural organization and characterization of the 5' flanking region. Genomics (1996) 1.63
Cross-protection against a lethal influenza virus infection by DNA vaccine to neuraminidase. Vaccine (2000) 1.63
Glycogen storage disease type III-hepatocellular carcinoma a long-term complication? J Hepatol (2006) 1.62
Expression of MAGE-antigens in normal tissues and cancer. Int J Cancer (2000) 1.60
SSX: a multigene family with several members transcribed in normal testis and human cancer. Int J Cancer (1997) 1.60
Nuclear-cytoplasmic transport and VAI RNA-independent translation of influenza viral messenger RNAs in late adenovirus-infected cells. Cell (1984) 1.57
Immunoreactivity for A103, an antibody to melan-A (Mart-1), in adrenocortical and other steroid tumors. Am J Surg Pathol (1998) 1.55
Measurement of cerebral hemodynamics with perfusion-weighted MR imaging: comparison with pre- and post-acetazolamide 133Xe-SPECT in occlusive carotid disease. AJNR Am J Neuroradiol (2001) 1.54
The usefulness of computed tomography in the diagnosis of impacted fish bones in the oesophagus. J Laryngol Otol (1998) 1.53
Caveolin-3 deficiency causes muscle degeneration in mice. Hum Mol Genet (2000) 1.53
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency: diagnosis by acylcarnitine analysis in blood. Am J Hum Genet (1993) 1.52
Limited association between a catechol-O-methyltransferase (COMT) polymorphism and breast cancer risk in Japan. Int J Clin Oncol (2001) 1.51
A103: An anti-melan-a monoclonal antibody for the detection of malignant melanoma in paraffin-embedded tissues. Am J Surg Pathol (1998) 1.51
Lack of evidence for an association between alpha-adducin and blood pressure regulation in Asian populations. Am J Hypertens (2000) 1.51
Genetic polymorphism in the 5'-flanking region of human CYP1A2 gene: effect on the CYP1A2 inducibility in humans. J Biochem (1999) 1.50
Development of Th1 and Th2 populations and the nature of immune responses to hepatitis B virus DNA vaccines can be modulated by codelivery of various cytokine genes. J Immunol (1998) 1.50
Genome-wide association study of bipolar I disorder in the Han Chinese population. Mol Psychiatry (2010) 1.49
Cutting edge: naturally occurring soluble form of mouse Toll-like receptor 4 inhibits lipopolysaccharide signaling. J Immunol (2000) 1.49
From dystrophinopathy to sarcoglycanopathy: evolution of a concept of muscular dystrophy. Muscle Nerve (1998) 1.48
Aspirin and angiotensin-converting enzyme inhibitors among elderly survivors of hospitalization for an acute myocardial infarction. Arch Intern Med (2001) 1.48
Variations in and correlates of length of stay in academic hospitals among patients with heart failure resulting from systolic dysfunction. Am J Manag Care (1999) 1.47
Caveolae in mesangial cells and caveolin expression in mesangial proliferative glomerulonephritis. Kidney Int (2001) 1.47
Molecular cloning of a novel member of the eukaryotic polypeptide chain-releasing factors (eRF). Its identification as eRF3 interacting with eRF1. J Biol Chem (1998) 1.47
Systemic correction of the muscle disorder glycogen storage disease type II after hepatic targeting of a modified adenovirus vector encoding human acid-alpha-glucosidase. Proc Natl Acad Sci U S A (1999) 1.46
Density of motility-related charge in the outer hair cell of the guinea pig is inversely related to best frequency. Neurosci Lett (1998) 1.45
NF-kappa B and STAT5 play important roles in the regulation of mouse Toll-like receptor 2 gene expression. J Immunol (2001) 1.45
Inactivation of the 14-3-3 sigma gene is associated with 5' CpG island hypermethylation in human cancers. Cancer Res (2000) 1.45
Improved fluorescence and dual color detection with enhanced blue and green variants of the green fluorescent protein. J Biol Chem (1998) 1.43
The putative glucose 6-phosphate translocase gene is mutated in essentially all cases of glycogen storage disease type I non-a. Eur J Hum Genet (1999) 1.42
CT10: a new cancer-testis (CT) antigen homologous to CT7 and the MAGE family, identified by representational-difference analysis. Int J Cancer (2000) 1.42
Gap junctions and cochlear homeostasis. J Membr Biol (2006) 1.41
Short capped hnRNA precursor chains in HeLa cells: continued synthesis in the presence of 5,6-dichloro-1-beta-D-ribofuranosylbenzimidazole. Biochemistry (1980) 1.41
Structural analysis of TL genes of the mouse. Proc Natl Acad Sci U S A (1985) 1.41
Direct and regulated interaction of integrin alphaEbeta7 with E-cadherin. J Cell Biol (1998) 1.40
NOx- concentrations in the rat hippocampus and striatum have no direct relationship to anaesthesia induced by ketamine. Br J Anaesth (2000) 1.40
Mutations in exon 3 of the glycogen debranching enzyme gene are associated with glycogen storage disease type III that is differentially expressed in liver and muscle. J Clin Invest (1996) 1.40
Identification of a tissue-specific putative transcription factor in breast tissue by serological screening of a breast cancer library. Cancer Res (2001) 1.39
Transcripts of the MHM region on the chicken Z chromosome accumulate as non-coding RNA in the nucleus of female cells adjacent to the DMRT1 locus. Chromosome Res (2001) 1.39
Glycogen storage disease types I and II: treatment updates. J Inherit Metab Dis (2007) 1.39
312-nanometer ultraviolet B light (narrow-band UVB) induces apoptosis of T cells within psoriatic lesions. J Exp Med (1999) 1.38
Brain abscess in glycogen storage disease type Ib. Acta Paediatr Scand (1991) 1.38
Molecular organization at the glycoprotein-complex-binding site of dystrophin. Three dystrophin-associated proteins bind directly to the carboxy-terminal portion of dystrophin. Eur J Biochem (1994) 1.38