about
Sterols and oxysterols in plasma from Smith-Lemli-Opitz syndrome patientsNeuroinflammation, mitochondrial defects and neurodegeneration in mucopolysaccharidosis III type C mouse model.Female mucopolysaccharidosis IIIA mice exhibit hyperactivity and a reduced sense of danger in the open field testCentral and haematopoietic interleukin-1 both contribute to ischaemic brain injury in miceRecET driven chromosomal gene targeting to generate a RecA deficient Escherichia coli strain for Cre mediated production of minicircle DNA.Genistein improves neuropathology and corrects behaviour in a mouse model of neurodegenerative metabolic diseaseRecommendations on clinical trial design for treatment of Mucopolysaccharidosis Type III.Neuropathology in mouse models of mucopolysaccharidosis type I, IIIA and IIIBHeparan sulfate inhibits hematopoietic stem and progenitor cell migration and engraftment in mucopolysaccharidosis I.Signal one and two blockade are both critical for non-myeloablative murine HSCT across a major histocompatibility complex barrier.An investigation of the middle and late behavioural phenotypes of Mucopolysaccharidosis Type-III.Assessment of sleep in children with mucopolysaccharidosis type III.Mucopolysaccharidosis type I, unique structure of accumulated heparan sulfate and increased N-sulfotransferase activity in mice lacking α-l-iduronidase.Hepatic stem cells: from inside and outside the liver?Sleep disordered breathing in mucopolysaccharidosis I: a multivariate analysis of patient, therapeutic and metabolic correlators modifying long term clinical outcome.Actigraphic investigation of circadian rhythm functioning and activity levels in children with mucopolysaccharidosis type III (Sanfilippo syndrome)Hematopoietic stem cell transplantation improves the high incidence of neutralizing allo-antibodies observed in Hurler's syndrome after pharmacological enzyme replacement therapy.Identification of age-dependent motor and neuropsychological behavioural abnormalities in a mouse model of Mucopolysaccharidosis Type IIEnzyme replacement therapy prior to haematopoietic stem cell transplantation in Mucopolysaccharidosis Type I: 10 year combined experience of 2 centres.Mucopolysaccharide diseases: a complex interplay between neuroinflammation, microglial activation and adaptive immunity.The role of antibodies in enzyme treatments and therapeutic strategies.Parental social support, coping strategies, resilience factors, stress, anxiety and depression levels in parents of children with MPS III (Sanfilippo syndrome) or children with intellectual disabilities (ID).Comparison of HIV- and EIAV-based vectors on their efficiency in transducing murine and human hematopoietic repopulating cells.Development of a self-assembling nuclear targeting vector system based on the tetracycline repressor protein.The fetal mouse is a sensitive genotoxicity model that exposes lentiviral-associated mutagenesis resulting in liver oncogenesis.In vivo T-cell depletion using alemtuzumab in family and unrelated donor transplantation for pediatric non-malignant disease achieves engraftment with low incidence of graft vs. host disease.Circadian rhythm and suprachiasmatic nucleus alterations in the mouse model of mucopolysaccharidosis IIIB.An araC-controlled bacterial cre expression system to produce DNA minicircle vectors for nuclear and mitochondrial gene therapy.Permanent phenotypic correction of hemophilia B in immunocompetent mice by prenatal gene therapy.Long-term transgene expression by administration of a lentivirus-based vector to the fetal circulation of immuno-competent mice.Analytical strategies for characterization of oxysterol lipidomes: liver X receptor ligands in plasma.Corrigendum to "Oncogenesis Following Delivery of a Nonprimate Lentiviral Gene Therapy Vector to Fetal and Neonatal Mice".Murine leukemia following irradiation conditioning for transplantation of lentivirally-modified hematopoietic stem cells.Is it congenital or acquired von Willebrands disease?Trial and error: how the unclonable human mitochondrial genome was cloned in yeast.Macrophage enzyme and reduced inflammation drive brain correction of mucopolysaccharidosis IIIB by stem cell gene therapy.Oncogenesis following delivery of a nonprimate lentiviral gene therapy vector to fetal and neonatal mice.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.Successful allogeneic bone marrow transplant for Niemann-Pick disease type C2 is likely to be associated with a severe 'graft versus substrate' effect.Quantitative charge-tags for sterol and oxysterol analysis.
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description
researcher
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wetenschapper
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հետազոտող
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name
Brian Bigger
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Brian Bigger
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Brian Bigger
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Brian Bigger
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Brian Bigger
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type
label
Brian Bigger
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Brian Bigger
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Brian Bigger
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Brian Bigger
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Brian Bigger
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prefLabel
Brian Bigger
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Brian Bigger
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Brian Bigger
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Brian Bigger
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Brian Bigger
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P108
P106
P1153
6602317807
P21
P31
P496
0000-0002-9708-1112