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Here we report the isolation of a murine model for heritable T cell lymphoblastic leukemia/lymphoma (T-ALL) called Spontaneous dominant leukemia (Sdl). Sdl heterozygous mice develop disease with a short latency and high penetrance; while mice homozygous for the mutation die early during embryonic de...
ORGANISM(S): Mus musculus 
Our group has proposed that low-density granulocytes (LDGs) play an important role in lupus pathogenesis, as they can damage endothelial cells and synthesize increased levels of proinflammatory cytokines and type I interferons. LDGs have a heightened capacity to synthesize neutrophil extracellular t...
ORGANISM(S): Homo sapiens 
PGC1beta is a transcriptional coactivator that potently stimulates mitochondrial biogenesis and respiration of cells. Here, we have generated mice lacking exons 3 to 4 of the Pgc1beta gene (PGC1beta E3,4-/E3,4- mice). These mice express a mutant protein that has reduced coactivation activity on a su...
ORGANISM(S): Mus musculus 
PGC-1alpha; is a coactivator of nuclear receptors and other transcription factors that regulates several metabolic processes, including mitochondrial biogenesis and respiration, hepatic gluconeogenesis, and muscle fiber-type switching. We show here that, while hepatocytes lacking PGC-1alpha; are def...
ORGANISM(S): Mus musculus 
Goal of the experiment: To identify correlated genes, pathways and groups of patients with systemic inflammatory response syndrome and septic shock that is indicative of biologically important processes active in these patients. Background: We measured gene expression levels and profiles of childr...
ORGANISM(S): Homo sapiens 
In an ongoing translational research program involving microarray-based expression profiles in pediatric septic shock, we have now conducted longitudinal studies focused on the temporal expression profiles of canonical signaling pathways and gene networks. Genome-level expression profiles were gener...
ORGANISM(S): Homo sapiens 
Genetic studies in T-cell acute lymphoblastic leukemia have uncovered a remarkable complexity of oncogenic and loss-of-function mutations. Amongst this plethora of genetic changes, NOTCH1 activating mutations stand out as the most frequently occurring genetic defect, identified in more than 50% of T...
ORGANISM(S): Homo sapiens 
The NOTCH signaling cascade, which is deregulated in T-cell acute lymphoblastic leukemia (T-ALL) and many other human cancers, offers an attractive target for molecular therapy. One approach employs gamma-secretase inhibitors (GSIs) to suppress production of the intracellular form of NOTCH (NICD), ...
ORGANISM(S): Homo sapiens 
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