PubRank

Brian W Bigger

Author PubWeight™ 24.69‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 The bone marrow functionally contributes to liver fibrosis. Gastroenterology 2006 2.52
2 Improved metabolic correction in patients with lysosomal storage disease treated with hematopoietic stem cell transplant compared with enzyme replacement therapy. J Pediatr 2009 1.52
3 Genistein improves neuropathology and corrects behaviour in a mouse model of neurodegenerative metabolic disease. PLoS One 2010 1.42
4 Permanent phenotypic correction of hemophilia B in immunocompetent mice by prenatal gene therapy. Blood 2004 1.30
5 Genistein reduces lysosomal storage in peripheral tissues of mucopolysaccharide IIIB mice. Mol Genet Metab 2009 1.26
6 Analytical strategies for characterization of oxysterol lipidomes: liver X receptor ligands in plasma. Free Radic Biol Med 2012 1.23
7 Plasma and urinary levels of dermatan sulfate and heparan sulfate derived disaccharides after long-term enzyme replacement therapy (ERT) in MPS I: correlation with the timing of ERT and with total urinary excretion of glycosaminoglycans. J Inherit Metab Dis 2012 1.09
8 Neuropathology in mouse models of mucopolysaccharidosis type I, IIIA and IIIB. PLoS One 2012 1.08
9 Heparin cofactor II-thrombin complex and dermatan sulphate:chondroitin sulphate ratio are biomarkers of short- and long-term treatment effects in mucopolysaccharide diseases. J Inherit Metab Dis 2010 1.01
10 Hematopoietic stem cell and gene therapy corrects primary neuropathology and behavior in mucopolysaccharidosis IIIA mice. Mol Ther 2012 1.00
11 Circadian rhythm and suprachiasmatic nucleus alterations in the mouse model of mucopolysaccharidosis IIIB. Behav Brain Res 2010 0.99
12 Evaluation of heparin cofactor II-thrombin complex as a biomarker on blood spots from mucopolysaccharidosis I, IIIA and IIIB mice. Mol Genet Metab 2009 0.97
13 Busulfan conditioning enhances engraftment of hematopoietic donor-derived cells in the brain compared with irradiation. Mol Ther 2013 0.97
14 Myeloid/Microglial driven autologous hematopoietic stem cell gene therapy corrects a neuronopathic lysosomal disease. Mol Ther 2013 0.95
15 Female mucopolysaccharidosis IIIA mice exhibit hyperactivity and a reduced sense of danger in the open field test. PLoS One 2011 0.93
16 Mucopolysaccharidosis type I, unique structure of accumulated heparan sulfate and increased N-sulfotransferase activity in mice lacking α-l-iduronidase. J Biol Chem 2011 0.93
17 Comparison of HIV- and EIAV-based vectors on their efficiency in transducing murine and human hematopoietic repopulating cells. Mol Ther 2005 0.91
18 Successful allogeneic bone marrow transplant for Niemann-Pick disease type C2 is likely to be associated with a severe 'graft versus substrate' effect. J Inherit Metab Dis 2010 0.90
19 Mucopolysaccharide diseases: a complex interplay between neuroinflammation, microglial activation and adaptive immunity. J Inherit Metab Dis 2013 0.89
20 Hematopoietic stem cell transplantation improves the high incidence of neutralizing allo-antibodies observed in Hurler's syndrome after pharmacological enzyme replacement therapy. Haematologica 2012 0.88
21 Murine leukemia following irradiation conditioning for transplantation of lentivirally-modified hematopoietic stem cells. Eur J Haematol 2007 0.78
22 Signal one and two blockade are both critical for non-myeloablative murine HSCT across a major histocompatibility complex barrier. PLoS One 2013 0.77
23 Characterisation of the T cell and dendritic cell repertoire in a murine model of mucopolysaccharidosis I (MPS I). J Inherit Metab Dis 2012 0.76