Brian C Mansfield

Author PubWeight™ 34.83‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Type I glycogen storage diseases: disorders of the glucose-6-phosphatase complex. Curr Mol Med 2002 1.96
2 Prevention of hepatocellular adenoma and correction of metabolic abnormalities in murine glycogen storage disease type Ia by gene therapy. Hepatology 2012 1.59
3 Multianalyte profiling of serum antigens and autoimmune and infectious disease molecules to identify biomarkers dysregulated in epithelial ovarian cancer. Cancer Epidemiol Biomarkers Prev 2008 1.54
4 Molecular mechanisms of neutrophil dysfunction in glycogen storage disease type Ib. Blood 2014 1.52
5 Impaired neutrophil activity and increased susceptibility to bacterial infection in mice lacking glucose-6-phosphatase-beta. J Clin Invest 2007 1.41
6 A glucose-6-phosphate hydrolase, widely expressed outside the liver, can explain age-dependent resolution of hypoglycemia in glycogen storage disease type Ia. J Biol Chem 2003 1.33
7 Glycogen storage disease type I and G6Pase-β deficiency: etiology and therapy. Nat Rev Endocrinol 2010 1.32
8 Complete normalization of hepatic G6PC deficiency in murine glycogen storage disease type Ia using gene therapy. Mol Ther 2010 1.21
9 Impaired glucose homeostasis, neutrophil trafficking and function in mice lacking the glucose-6-phosphate transporter. Hum Mol Genet 2003 1.15
10 Neutrophil stress and apoptosis underlie myeloid dysfunction in glycogen storage disease type Ib. Blood 2008 1.11
11 Gene therapy for type I glycogen storage diseases. Curr Gene Ther 2007 1.07
12 Brain contains a functional glucose-6-phosphatase complex capable of endogenous glucose production. J Biol Chem 2005 1.06
13 Mutations in the glucose-6-phosphatase-alpha (G6PC) gene that cause type Ia glycogen storage disease. Hum Mutat 2008 1.03
14 The glucose-6-phosphate transporter is a phosphate-linked antiporter deficient in glycogen storage disease type Ib and Ic. FASEB J 2008 1.03
15 Development and preliminary evaluation of a multivariate index assay for ovarian cancer. PLoS One 2009 1.02
16 SLC37A1 and SLC37A2 are phosphate-linked, glucose-6-phosphate antiporters. PLoS One 2011 1.01
17 Neutropenia in type Ib glycogen storage disease. Curr Opin Hematol 2010 0.98
18 Sustained hepatic and renal glucose-6-phosphatase expression corrects glycogen storage disease type Ia in mice. Hum Mol Genet 2002 0.97
19 Bone marrow-derived cells require a functional glucose 6-phosphate transporter for normal myeloid functions. J Biol Chem 2006 0.97
20 Lack of glucose recycling between endoplasmic reticulum and cytoplasm underlies cellular dysfunction in glucose-6-phosphatase-beta-deficient neutrophils in a congenital neutropenia syndrome. Blood 2010 0.96
21 Necrotic foci, elevated chemokines and infiltrating neutrophils in the liver of glycogen storage disease type Ia. J Hepatol 2007 0.92
22 A potential new role for muscle in blood glucose homeostasis. J Biol Chem 2004 0.92
23 Comprehensive serum profiling for the discovery of epithelial ovarian cancer biomarkers. PLoS One 2011 0.91
24 Glucose-6-phosphatase-β, implicated in a congenital neutropenia syndrome, is essential for macrophage energy homeostasis and functionality. Blood 2012 0.87
25 Recombinant AAV-directed gene therapy for type I glycogen storage diseases. Expert Opin Biol Ther 2011 0.85
26 G-CSF improves murine G6PC3-deficient neutrophil function by modulating apoptosis and energy homeostasis. Blood 2011 0.83
27 Normoglycemia alone is insufficient to prevent long-term complications of hepatocellular adenoma in glycogen storage disease type Ib mice. J Hepatol 2009 0.82
28 The islet-specific glucose-6-phosphatase-related protein, implicated in diabetes, is a glycoprotein embedded in the endoplasmic reticulum membrane. FEBS Lett 2004 0.82
29 The upstream enhancer elements of the G6PC promoter are critical for optimal G6PC expression in murine glycogen storage disease type Ia. Mol Genet Metab 2013 0.80
30 The signature motif in human glucose-6-phosphate transporter is essential for microsomal transport of glucose-6-phosphate. Hum Genet 2003 0.79
31 Type I glycogen storage diseases: disorders of the glucose-6-phosphatase/glucose-6-phosphate transporter complexes. J Inherit Metab Dis 2014 0.77
32 Oxidative stress mediates nephropathy in type Ia glycogen storage disease. Lab Invest 2010 0.77
33 The SLC37 family of phosphate-linked sugar phosphate antiporters. Mol Aspects Med 2013 0.76
34 A method for assessing and maintaining the reproducibility of mass spectrometric analyses of complex samples. Rapid Commun Mass Spectrom 2009 0.75
35 A method for monitoring and controlling reproducibility of intensity data in complex electrospray mass spectra: a thermometer ion-based strategy. J Am Soc Mass Spectrom 2008 0.75