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[PDF] Top 20 Glucocerebrosidase deficiency and mitochondrial impairment in experimental Parkinson disease

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Glucocerebrosidase deficiency and mitochondrial impairment in experimental Parkinson disease

Glucocerebrosidase deficiency and mitochondrial impairment in experimental Parkinson disease

... Gaucher disease is an autosomal recessive disease, caused by a lack or functional deficiency of the lysosomal enzyme, glucocerebrosidase ...mutations in the glucocerebrosidase ... Voir le document complet

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Glucocerebrosidase deficiency dramatically impairs human bone marrow haematopoiesis in an in vitro model of Gaucher disease.

Glucocerebrosidase deficiency dramatically impairs human bone marrow haematopoiesis in an in vitro model of Gaucher disease.

... enzyme deficiency consist principally in ...thrombocytopenia and 37% with anaemia (Pastores, et al 2004, Weinreb, et al 2002), whilst leucopoenia is rare, occurs later in life and is ... Voir le document complet

25

Bee Venom and Its Component Apamin as Neuroprotective Agents in a Parkinson Disease Mouse Model

Bee Venom and Its Component Apamin as Neuroprotective Agents in a Parkinson Disease Mouse Model

... result in significant impairment of motor parameters in MPTP/probenecid- treated ...loss and accompanying striatal DA depletion is insufficient to induce a behavioural ...deficits in ... Voir le document complet

9

Dysfunction of mitochondrial Lon protease and identification of oxidized protein in mouse brain following exposure to MPTP: Implications for Parkinson disease

Dysfunction of mitochondrial Lon protease and identification of oxidized protein in mouse brain following exposure to MPTP: Implications for Parkinson disease

... handled, and cared for in accordance with the Guide for the Care and Use of Laboratory Animals (NCR [National Research Council] 1996) and European Union Council Directive 86/609/EEC, ... Voir le document complet

45

Altered expression of vesicular glutamate transporters VGLUT1 and VGLUT2 in Parkinson disease.

Altered expression of vesicular glutamate transporters VGLUT1 and VGLUT2 in Parkinson disease.

... studies in humans and animal models have reported that nigro-striatal dopaminergic depletion over-activates the cortico-striatal pathway ...[7,8]. In experimental models of PD, dopamine ... Voir le document complet

20

Understanding Dopaminergic Cell Death Pathways in Parkinson Disease: Cell Death in Parkinson Disease

Understanding Dopaminergic Cell Death Pathways in Parkinson Disease: Cell Death in Parkinson Disease

... B., and Halliday, G.M. (2014). Reduced glucocerebrosidase is associated with increased α- synuclein in sporadic Parkinson's ...drives mitochondrial fission by the Parkinson ... Voir le document complet

58

Transcriptome analysis reveals link between proteasomal and mitochondrial pathways in Parkinson's disease.

Transcriptome analysis reveals link between proteasomal and mitochondrial pathways in Parkinson's disease.

... Parkinson's disease cases using a whole-tissue approach ...Parkinson's disease is not a disease of one specific cell type ...cells and neurons both within and beyond the ...from ... Voir le document complet

11

AIF Deficiency Induces Early Mitochondrial Degeneration in Brain Followed by Progressive Multifocal Neuropathology

AIF Deficiency Induces Early Mitochondrial Degeneration in Brain Followed by Progressive Multifocal Neuropathology

... Hence, mitochondrial degeneration is rather a cause than a consequence of the degenerating process, even if we can not exclude that this latter might enhance in turn mitochondrial ...findings, ... Voir le document complet

21

Subthalamus stimulation in Parkinson disease: Accounting for the bilaterality of contacts

Subthalamus stimulation in Parkinson disease: Accounting for the bilaterality of contacts

... improvement in Dopa‑OFF condition was 37.1% [Table 1b]. Disease severity increased over 1 year, with UPDRS III score in DopaOFF‑StimOFF conditions varying from ...(52.8%), and high, >50%, ... Voir le document complet

24

GABAergic inhibition in dual-transmission cholinergic and GABAergic striatal interneurons is abolished in Parkinson disease

GABAergic inhibition in dual-transmission cholinergic and GABAergic striatal interneurons is abolished in Parkinson disease

... failure in CGINs contribute to motor deficiency produced by dopamine ...observed in many neuronal systems and shown to modulate integrative ...excitatory and inhibitory drives and exert ... Voir le document complet

15

Proton MR Spectroscopy for Diagnosis and Evaluation of Treatment Efficacy in Parkinson Disease

Proton MR Spectroscopy for Diagnosis and Evaluation of Treatment Efficacy in Parkinson Disease

... tCho, and m-Ins. Previous 1.5-T MR spectroscopic studies in different brain structures of PD patients have reported conflicting results, likely ex- plained by nonhomogenous patient groups ...moderate ... Voir le document complet

9

AMP-activated protein kinase alpha2 deficiency affects cardiac cardiolipin homeostasis and mitochondrial function.

AMP-activated protein kinase alpha2 deficiency affects cardiac cardiolipin homeostasis and mitochondrial function.

... selective deficiency of AMPK α 2 causes a significant decrease in maximal mitochondrial respiration, whether carbohydrates or lipids were used as ...defect in the function of complex-I of the ... Voir le document complet

28

Verbal Learning in Alzheimer’s disease and Mild Cognitive Impairment: neuroanatomic correlates of acquisition and consolidation performances

Verbal Learning in Alzheimer’s disease and Mild Cognitive Impairment: neuroanatomic correlates of acquisition and consolidation performances

... impaired in AD patients even at a very early stage of the disease ...changes in the hippocampal formation. In contrast, variations of relatively intact performances of controls and ... Voir le document complet

1

OXIDATIVE STRESS AND MITOCHONDRIAL DYNAMICS MALFUNCTION ARE LINKED IN PELIZAEUS-MERZBACHER DISEASE

OXIDATIVE STRESS AND MITOCHONDRIAL DYNAMICS MALFUNCTION ARE LINKED IN PELIZAEUS-MERZBACHER DISEASE

... scanned and quantified using a GS800 Densitometer and Quantity One software ...electrophoresis and Western blotting, frozen spi- nal cords were homogenized in a lysis buffer (180 mM KCl, 5 mM ... Voir le document complet

20

The impact of apple peel polyphenols on intestinal and mitochondrial functions in experimental colitis

The impact of apple peel polyphenols on intestinal and mitochondrial functions in experimental colitis

... (200 and 400 mg/kg/day) is not to examine the dose response, but rather to understand how mice with DSS-induced experimental UC respond to physiological and supraphysiological ...better in ... Voir le document complet

145

Automated MRI measures identify individuals with mild cognitive impairment and Alzheimer's disease

Automated MRI measures identify individuals with mild cognitive impairment and Alzheimer's disease

... examined in this ...obtained in accor- dance with guidelines of the Washington University Human Studies Committee (St Louis, ...published in previous studies (Salat et ...(BIRN) and the ... Voir le document complet

11

Axial symptoms predict mortality in patients with Parkinson disease with subthalamic stimulation

Axial symptoms predict mortality in patients with Parkinson disease with subthalamic stimulation

... severity in predicting mortality, the HRs for associa- tions between akinesia and rigidity and mortality are greater than ...akinesia and rigidity symptoms is substantively asso- ciated with ... Voir le document complet

14

Thiamine Deficiency--Induced Partial Necrosis and Mitochondrial Uncoupling in Neuroblastoma Cells Are Rapidly Reversed by Addition of Thiamine

Thiamine Deficiency--Induced Partial Necrosis and Mitochondrial Uncoupling in Neuroblastoma Cells Are Rapidly Reversed by Addition of Thiamine

... P olarographic recording of oxygen consumption by neu- roblastoma cells in whole culture media containing various amounts of thiamine (Α), TD medium (Β), TDA medium (C), and TDA cells af[r] ... Voir le document complet

7

e-Cadherin in 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine-Induced Parkinson Disease

e-Cadherin in 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine-Induced Parkinson Disease

... 3- and 5-day intervals and showed a significant reduction in the number of neurons in the substantia nigra pars compacta (SNpc) and striatal dopamine levels ...duration, and the ... Voir le document complet

9

Arabidopsis thaliana alternative dehydrogenases: a potential therapy for mitochondrial complex I deficiency? Perspectives and pitfalls

Arabidopsis thaliana alternative dehydrogenases: a potential therapy for mitochondrial complex I deficiency? Perspectives and pitfalls

... fragmented and reverse transcribed with the Elute, Prime and Fragment Mix ...ligation and library enrichment were performed as described in the Low Throughput protocol of the TruSeq Stranded ... Voir le document complet

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