Defects in ribosomal biogenesis profoundly affect organismal development and cellular function, and these ribosomopathies produce a variety of phenotypes. One ribosomopathy, Shwachman-Diamond syndrome (SDS) is characterized by neutropenia, pancreatic exocrine insufficiency, and skeletal anomalies. SDS results from biallelic mutations in , which encodes a ribosome assembly factor.
View Article and Find Full Text PDFProstaglandin E (PGE ) has been implicated in counteracting fibroblast differentiation by TGFβ1 during pulmonary fibrosis. However, the precise mechanism is not well understood. We show here that PGE via EP R and EP R inhibits the expression of mechanosensory molecules Lysyl Oxidase Like 2 (LOXL2), myocardin-related transcription factor A (MRTF-A), ECM proteins, plasminogen activation inhibitor 1 (PAI-1), fibronectin (FN), α-smooth muscle actin (α-SMA), and redox sensor (nicotinamide adenine dinucleotide phosphate (NADPH) oxidase 4 (NOX4)) required for TGFβ1-mediated fibroblast differentiation.
View Article and Find Full Text PDFShwachman-Diamond syndrome (SDS) is characterized by exocrine pancreatic insufficiency, neutropenia, and skeletal abnormalities. Biallelic mutations in SBDS, which encodes a ribosome maturation factor, are found in 90% of SDS cases. Sbds-/- mice are embryonic lethal.
View Article and Find Full Text PDFCancer Genet Cytogenet
November 1987
J Exp Psychol Anim Behav Process
October 1985
An adjusting procedure was used to measure pigeons' preferences among alternatives that differed in the duration of a delay before reinforcement and of an intertrial interval (ITI) after reinforcement. In most conditions, a peck at a red key led to a fixed delay, followed by reinforcement, a fixed ITI, and then the beginning of the next trial. A peck at a green key led to an adjustable delay, reinforcement, and then the next trial began without an ITI.
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