Search results for "HeLa"
showing 10 items of 738 documents
Compound heterozygosity in the SPG4 gene causes hereditary spastic paraplegia
2008
The SPG4 gene is frequently mutated in autosomal dominant form of hereditary spastic paraplegia (HSP). We report that the compound heterozygous sequence variants S44L, a known polymorphism, and c.1687G>A, a novel mutation in SPG4 cause a severe form of HSP in a patient. The family members carrying solely c.1687G>A mutation are asymptomatic for HSP. The reverse transcriptase-polymerase chain reaction (RT-PCR) analysis revealed that the c.1687G>A mutation is a splice site mutation and causes skipping of the exon 15 of spastin. Furthermore, quantification of RT-PCR products by sequencing and quantification of allele-specific expression by pyrosequencing assay revealed that c.1687G>A is a leaky…
FM19G11, a New Hypoxia-inducible Factor (HIF) Modulator, Affects Stem Cell Differentiation Status
2009
The biology of the alpha subunits of hypoxia-inducible factors (HIF alpha) has expanded from their role in angiogenesis to their current position in the self-renewal and differentiation of stem cells. The results reported in this article show the discovery of FM19G11, a novel chemical entity that inhibits HIF alpha proteins that repress target genes of the two alpha subunits, in various tumor cell lines as well as in adult and embryonic stem cell models from rodents and humans, respectively. FM19G11 inhibits at nanomolar range the transcriptional and protein expression of Oct4, Sox2, Nanog, and Tgf-alpha undifferentiating factors, in adult rat and human embryonic stem cells, FM19G11 activit…
Zinc chelation during non-lesioning overexcitation results in neuronal death in the mouse hippocampus
2003
In the hippocampus, chelatable zinc is accumulated in vesicles of glutamatergic presynaptic terminals, abounding specially in the mossy fibers, from where it is released with activity and can exert a powerful inhibitory action upon N-methyl-D-aspartate receptors. Zinc is therefore in a strategic situation to control overexcitation at the zinc-rich excitatory synapses, and consequently zinc removal during high activity might result in excitotoxic neuronal damage. We analyzed the effect of zinc chelation with sodium dietyldithiocarbamate under overexcitation conditions induced by non-lesioning doses of kainic acid in the mouse hippocampus, to get insight into the role of zinc under overexcita…
Neural overexcitation and implication of NMDA and AMPA receptors in a mouse model of temporal lobe epilepsy implying zinc chelation.
2006
Summary: Purpose: Zinc chelation with diethyldithiocarbamate (DEDTC) during nondamaging kainic acid administration enhances excitotoxicity to the level of cell damage. The objective of this work was to study the developing of the lesion in this model of temporal lobe epilepsy and the implications of the different types of glutamate receptors. Methods: The antagonist of the N-methyl-d-aspartate (NMDA) receptor MK-801, and the antagonist of the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionate (AMPA) receptor GYKI52466, were used concomitantly with intraperitoneal administration of kainic acid (15 mg/kg) followed by DEDTC (150 mg/kg) in mouse. The animals were killed at different times from 4 …
Effect of acidosis and anoxia on iron delocalization from brain homogenates.
1992
Abstract Cortical homogenates were prepared from rat brain in Krebs-Ringer phosphate media adjusted to pH 7, 6 or 5 and incubated for 1 hr under aerotic or anaerobic conditions in the presence of dipyridyl, an iron chelator. Low molecular weight species (LMWS) iron was measured spectrophotometrically after passing of the homogenates through a 10,000- M , ultrafiltration membrane. Following aerobic incubation, LMWS iron reached 1.24 μg/g tissue at pH 7, and increased 1.7-fold at pH 6 and 3.1-fold at pH 5. Anoxia enhanced significantly the amount of ultrafiltrable iron at the three pH values, the LMWS iron level being increased by 190% at pH 7, by 113% at pH 6, and by 77% at pH 5. Addition of…
Cardiac and hepatic iron and ejection fraction in thalassemia major: Multicentre prospective comparison of combined Deferiprone and Deferoxamine ther…
2013
Background: Due to the limited data available in literature, the aim of this multi-centre study was to prospectively compare in thalassemia major (TM) patients the efficacy of combined deferiprone (DFP) and deferoxamine (DFO) regimen versus either DFP and DFO in monotherapy by cardiovascular magnetic resonance (CMR) over a follow up of 18 months. Methods: Among the first 1135 TM patients in the MIOT (Myocardial Iron Overload in Thalassemia) network, we evaluated those who had received either combined regimen (DFO + DFP, N=51) or DFP (N=39) and DFO (N=74) monotherapies between the two CMR scans. Iron overload was measured by T2* multiecho technique. Biventricular function parameters were qua…
Evaluation of demineralized bone and bone transplantsin vitroandin vivowith cone beam computed tomography imaging
2010
The objective of this study was to determine the ability of two flat panel cone beam CT (CBCT) devices to identify demineralized bone and bone transplants in vivo and in vitro.Datasets from patients with autologous bone grafts (n = 9, KaVo 3DeXam (KaVo, Biberach, Germany); n = 38, Accuitomo 40 (Morita, Osaka, Japan)) were retrospectively evaluated. Demineralized and non-demineralized porcine cancellous bone blocks were examined with the two CBCT devices. A SawBone skull (Pacific Research Laboratories, Vashon, WA) was used as a positioning tool for the bone blocks. Descriptive evaluation and image quality assessment were conducted on the KaVo 3DeXam data (voxel size 0.3 mm) using the OsiriX …
Purification of a glucose-binding protein from rat liver nuclei. Evidence for a role in targeting of nuclear mRNP to nuclear pore complex.
1992
A nuclear carbohydrate-binding protein with a molecular mass of 67 kDa (CBP67), which is specific for glucose residues, was purified to essential homogeneity from rat liver nuclear extracts. This protein could also be isolated from nuclear ribonucleoprotein (RNP) complexes by extraction in the presence of 0.6 M or 2 M NaCl, but it was absent in polysomal RNP complex. The binding of the purified protein, which has an isoelectric point of 7.3, to glucose-containing glycoconjugates depends on the presence of Ca2+ and Mg2+. Using closed nuclear envelope vesicles as a system to study nuclear transport of RNA, it was shown that both entrapped polysomal mRNA and nuclear RNA precursors are readily …
Complexity of the Hereditary Motor and Sensory Neuropathies
2015
Early-onset hereditary motor and sensory neuropathies are rare diseases representing a broad clinical and genetic spectrum. Without a notable familial history, the clinical diagnosis is complicated because acquired causes of peripheral neuropathy, such as inflammatory neuropathies, neuropathies with toxic causes, and nutritional deficiencies, must be considered. We examined the clinical, electrophysiological, and pathologic manifestations of a boy with an initial diagnosis of chronic inflammatory demyelinating polyneuropathy. The progression of the disease despite treatment led to a suspicion of hereditary motor and sensory neuropathy. Genetic testing revealed the presence of the MPZ p.D90…
Beneficial effect of dipyridyl, a liposoluble iron chelator against focal cerebral ischemia: In vivo and in vitro evidence of protection of cerebral …
2007
Whereas iron chelators were shown to induce neuroprotection against brain injury, the effect of iron chelators on ischemia-induced damage of cerebral endothelium is largely unknown. Our objective was to explore the endothelioprotective effect of the lipophilic iron chelator dipyridyl (DP) (i) in vitro on the death of cerebral endothelial cells (CECs) subjected to intracellular iron loading and (ii) in vivo on the ischemia-induced blood-brain barrier (BBB) disruption. When given shortly after iron exposure or brain ischemia, DP prevented the death of CECs and diminished BBB disruption, respectively, whereas a delayed administration of DP was associated with a lower CECs protection. Interesti…