Cp/Heph mutant mice have iron-induced neurodegeneration diminished by deferiprone
نویسندگان
چکیده
منابع مشابه
Bone resorption induced by parathyroid hormone is strikingly diminished in collagenase-resistant mutant mice.
Parathyroid hormone (PTH) stimulates bone resorption by acting directly on osteoblasts/stromal cells and then indirectly to increase differentiation and function of osteoclasts. PTH acting on osteoblasts/stromal cells increases collagenase gene transcription and synthesis. To assess the role of collagenase in the bone resorptive actions of PTH, we used mice homozygous (r/r) for a targeted mutat...
متن کاملP 97: Neurodegeneration Induced by Tau protein
Tau is one of several types of microtubule-associated proteins (MAPs), responsible for the assembly and stability of microtubule networks that is present only in neurons and predominantly localized in axons which its functions are tightly regulated by phosphorylation. Via as yet unknown mechanisms, tau becomes hyperphosphorylated and accompanies with neuronal degeneration, loss of synapses...
متن کاملA pilot trial of deferiprone for neurodegeneration with brain iron accumulation.
Deferiprone was shown to reverse iron deposition in Friedreich's ataxia. This multi-center, unblinded, single-arm pilot study evaluated safety and efficacy of deferiprone for reducing cerebral iron accumulation in neurodegeneration with brain iron accumulation. Four patients with genetically-confirmed pantothenate kinase-associated neurodegeneration, and 2 with parkinsonism and focal dystonia, ...
متن کاملThe oral iron chelator deferiprone protects against systemic iron overload-induced retinal degeneration in hepcidin knockout mice.
PURPOSE To investigate the retinal-protective effects of the oral iron chelator deferiprone (DFP) in mice lacking the iron regulatory hormone hepcidin (Hepc). These Hepc knockout (KO) mice have age-dependent systemic and retinal iron accumulation leading to retinal degeneration. METHODS Hepc KO mice were given DFP in drinking water from age 6 to 18 months. They were then compared to Hepc KO m...
متن کاملp 97: neurodegeneration induced by tau protein
tau is one of several types of microtubule-associated proteins (maps), responsible for the assembly and stability of microtubule networks that is present only in neurons and predominantly localized in axons which its functions are tightly regulated by phosphorylation. via as yet unknown mechanisms, tau becomes hyperphosphorylated and accompanies with neuronal degeneration, loss of synapses, abe...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Journal of Neurochemistry
سال: 2015
ISSN: 0022-3042
DOI: 10.1111/jnc.13292