Implications of a peroxisome proliferator-activated receptor alpha (PPARα) ligand clofibrate in breast cancer.
North Chicago, United States. In Oncotarget, Dec 2015
Surprisingly, the expression of lipogenic pathway genes including SREBP-1c (sterol regulatory element-binding protein-1c), HMG-CoA synthase, SPTLC1 (serine palmitoyltransferase long-chain), and Acyl-CoA oxidase (ACO) decreased with a concurrent increase in fatty acid oxidation genes such as CPT-1a (carnitine palmitoyltransferase 1a) and SREBP-2 (Sterol regulatory element-binding protein-2).
Mitochondrial dynamics and inherited peripheral nerve diseases.
Milano, Italy. In Neurosci Lett, Jul 2015
Mutations in several proteins fundamental for the axonal transport or forming the axonal cytoskeleton result in peripheral neuropathy, i.e., CMT, distal hereditary motor neuropathy (dHMN) or hereditary sensory and autonomic neuropathy (HSAN), as well as in hereditary spastic paraplegia.
The shifting paradigm of Charcot-Marie-Tooth disease.
Strasbourg, France. In Rev Neurol (paris), Jun 2015
The phenotypic spectrum of CMT overlaps with other inherited neuropathies such as distal hereditary motor neuropathy (dHMN), hereditary sensory and autonomic neuropathy (HSAN), spinal muscular atrophy (SMA) subtypes, and the neuropathies of mitochondrial disorders.
The consequences of genetic and pharmacologic reduction in sphingolipid synthesis.
Dallas, United States. In J Inherit Metab Dis, 2015
Complete synthetic blockage may be lethal if it includes all sphingolipids, such as in a global knockout of serine palmitoyltransferase. Partial inhibition of sphingolipid synthetic pathways is usually benign and may have beneficial effects in a number of lysosomal diseases and in more common pathologies, as seen in animal models for atherosclerosis, polycystic kidney disease, diabetes, and asthma.
Orm family proteins mediate sphingolipid homeostasis.
San Francisco, United States. In Nature, 2010
Starting from an unbiased functional genomic approach in Saccharomyces cerevisiae, we identify Orm proteins as negative regulators of sphingolipid synthesis that form a conserved complex with serine palmitoyltransferase, the first and rate-limiting enzyme in sphingolipid production.