Neurological Laboratory of Hebei Province

Shijiazhuang, China

Neurological Laboratory of Hebei Province

Shijiazhuang, China
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Hong K.,Hebei Medical University | Li Y.,Hebei Medical University | Li Y.,Institute of Cardiocerebrovascular Disease | Li Y.,Neurological Laboratory of Hebei Province | And 11 more authors.
Neuroscience Letters | Year: 2012

TAR DNA binding protein of 43. kDa (TDP-43), which has been associated with amyotrophic lateral sclerosis (ALS), plays an essential role in neurodegenerative disease pathogenesis. In particular, mitochondrial dysfunction is involved in the disease development. Thus, we investigated how TDP-43 is related to mitochondrial dysfunction. In this study, we found that overexpression of TDP-43 and its C-terminal fragments resulted in mitochondrial damage. In addition, full-length TDP-43 and truncated TDP-43 were localized in the mitochondria, where autophagy was activated, indicated by changes of LC3-II and p62. These studies suggest that human TDP-43 and its C-terminal fragments may cause mitochondrial dysfunction and enhance mitophagy. © 2012 Elsevier Ireland Ltd.

Jia L.-J.,Hebei Medical University | Jia L.-J.,Institution of Cardiocerebrovascular Disease | Jia L.-J.,Neurological Laboratory of Hebei Province | Qu Z.-Z.,Hebei Medical University | And 5 more authors.
BMC Neurology | Year: 2017

Background: Uremic Encephalopathy (UE) is a neurological complication associated with acute or chronic renal failure. Imaging findings of UE may present involvement of the basal ganglia, cortical or subcortical regions, and white matter. We report a rare case of UE caused by neurogenic bladder with isolated brainstem involvement revealed by magnetic resonance imaging (MRI). Immediate therapy resulted in full recovery of neurological signs and changes on MRI. Case presentation: A 14-year-old Han Chinese woman with a history of chronic renal failure caused by neurogenic bladder. On admission, she was unconscious and her pupils presented different sizes, while her vital signs were normal. MRI showed high signal in the dorsal pontine base and in the mid brain on fluid-attenuated inversion-recovery (FLAIR) imaging and on T2-weighted imaging while the signal was normal on diffusion-weighted images (DWI). Blood analysis revealed renal failure and acidosis. After urinary retention treatment and acidosis correction, the patient soon recovered. Follow-up MRI 2 months after the discharge revealed complete resolution of UE in the brainstem. Conclusion: We reported a rare case of a patient with UE that had unusual imaging manifestations for whom timely diagnosis and treatment assured recovery. © 2017 The Author(s).

Liu Y.,Hebei Medical University | Duan W.,Hebei Medical University | Duan W.,Institute of Cardiocerebrovascular Disease | Duan W.,Neurological Laboratory of Hebei Province | And 12 more authors.
Neuroscience | Year: 2014

The C-terminal fragments-25(CTF25) of TDP-43 is a fragment of TAR DNA-binding protein 43. kDa (TDP-43), which is involved in RNA metabolism, neurite outgrowth, and neuronal development and stress granules. Not until recently did evidence suggest that CTF25 might play an important role in amyotrophic lateral sclerosis (ALS) pathogenesis. However, mechanical details on CTF25 causing motor neuron degeneration still remain unknown. To study the toxicity of CTF25 of TDP-43, we established a cellular model stably expressing CTF25 of TDP-43. Herein, we found that stably expressed CTF25 could induce significant oxidative stress and was mainly degraded by the proteasome pathway in cells. Furthermore, the neurotoxicity of CTF25 of TDP-43 was dependent on proteasome activity. In addition, electron microscopy showed mitochondrial swelling and cristae dilation in cells expressing CTF25 and that CTF25 aggregates were characterized by filamentous bundles and electron dense granular material. In conclusion, the new cellular model mimics classical toxic TDP-43 cellular model and interestingly the toxicity of CTF25 is dependent on the proteasome. © 2014 IBRO.

Duan W.,Hebei Medical University | Duan W.,Institute of Cardiocerebrovascular | Duan W.,Neurological Laboratory of Hebei Province | Guo Y.,Hebei Medical University | And 11 more authors.
Molecular Neurobiology | Year: 2014

Mutation of TAR DNA-binding protein-43 (TDP-43) was detected in familiar and sporadic amyotrophic lateral sclerosis, and pathological TDP-43 was identified in the frontotemporal lobar degeneration. The neuroprotective functions of curcumin derivatives were assessed in motor neurons transfected with mutant TDP-43. We found that curcumin derivatives reduced the levels of TDP-43 fragments. Furthermore, we evaluated these compounds on the cellular model that the cells were transfected with TDP-25. We found that the expression level and aggregate formation of TDP-25 were significantly reduced by monocarbonyl dimethoxycurcumin C (Compound C). To study on the neuroprotective functions of curcumin derivatives, the neuroblastoma-spinal cord-34 cells transfected with mutant TDP-43 were assessed by the level of lactate dehydrogenase (LDH) and malondialdehyde bisdimethyl acetal (MDA) that were involved in the oxidative stress. We found that Compound C ameliorated the damage of mutant TDP-43 by reducing the level of MDA and LDH. Furthermore, heme oxygenase-1 (HO-1) was induced by Compound C significantly higher than other compounds. Znpp, which is known an inhibitor of HO-1, dramatically interfered with the function of Compound C. In addition, Compound C was tested in vivo, and HO-1 was significantly upregulated at the hippocampus. These findings suggest that Compound C, which degrades TDP-43 fragment and strengthens the antioxidant ability by HO-1, is a promising agent for TDP-43 proteinopathy. © 2013 Springer Science+Business Media New York.

Li Z.,Hebei Medical University | Li Z.,Institute of Cardiocerebrovascular Disease | Li Z.,Neurological Laboratory of Hebei Province | Duan W.,Hebei Medical University | And 9 more authors.
Neuroscience Letters | Year: 2017

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease lacking of curable treatments to date. Adeno-associated virus (AAV) vectors make gene therapy an effective strategy in treating neurological disorders. Despite Kaspar and colleagues have showed that AAV-IGF1 delivery successfully prolonged the survival of SOD1G93A mice, whether IGF-1 act as a protective role in the TDP-43 mutant model still have not been reported. In this study, we proved that AAV9 vector mediated expression of human wild-type IGF-1 protected TDP-25 cells from apoptosis and oxidative stress. Furthermore, we demonstrated that the hIGF-1 exerted its neuroprotective effect through an Akt-dependent manner in cultured motoneurons. We also described a hypothesis that in cultured TDP-25-associated degeneration of motoneurons, the neuronal survival promotion of IGF-1 was related to VEGF. © 2017 Elsevier B.V.

Sun C.,Hebei Medical University | Liu Y.,Hebei Medical University | Liu Y.,Institute of Cardiocerebrovascular Disease | Liu Y.,Neurological Laboratory of Hebei Province | And 5 more authors.
Brain Research Bulletin | Year: 2017

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease involving motor neurons in the motor cortex, brainstem and spinal cord. ALS leads to progressive, aggravated muscle weakness and paralysis. Although the precise pathogenesis remains unknown, several studies have shown that estrogens exert neuroprotective effects during the course of the disease. Aromatase is the key enzyme in estrogen synthesis. In the present study, we used immunohistochemistry, immunofluorescence and western blotting to observe the characteristics of aromatase expression in the spinal cords of copper-zinc superoxide dismutase-1 (SOD1)-G93A transgenic mice. Under normal and nearly normal (pre-symptomatic stage) conditions, the motor neurons in the spinal anterior horn expressed aromatase. After disease onset, astrocytes began to express aromatase. The total level of aromatase expression decreased with disease progression. These findings may provide the basis for the pathogenesis of ALS through glial aromatization during the progression of this disease. © 2017 Elsevier Inc.

Guo Y.,Hebei Medical University | Guo Y.,Institute of Cardiocerebrovascular Disease | Guo Y.,Neurological Laboratory of Hebei Province | Zhang K.,Hebei Medical University | And 12 more authors.
Brain Research | Year: 2011

Diallyl trisulfide (DATS) is one of the major constituents in garlic oil and has been documented to transcriptionally activate phase II enzymes. The purpose of this study is to evaluate the effects of DATS in prolonging disease duration and survival in a transgenic mouse model of amyotrophic lateral sclerosis (ALS). SOD1-G93A transgenic mice were randomly divided into DATS-treated group (80 mg/kg/d, p.o.) and vehicle-treated group at disease onset stage. Oral administration of DATS beginning at clinical onset stage significantly prolonged disease duration and extended life span for about one week. DATS treatment induced HO-1 and reduced GFAP expression in the lumbar spinal cord of SOD1-G93A transgenic mice. This study indicates that DATS has multifunctional neuroprotective effects in SOD1-G93A transgenic mice. © 2010 Elsevier B.V. © 2011 Elsevier B.V. All rights reserved.

Han H.,Hebei Medical University | Wei W.,Hebei Medical University | Duan W.,Hebei Medical University | Duan W.,Institute of Cardiocerebrovascular Disease | And 14 more authors.
In Vitro Cellular and Developmental Biology - Animal | Year: 2015

Autophagy-linked FYVE (Alfy) is a protein implicated in the selective degradation of aggregated proteins. In our present study, we found that Alfy was recruited into the aggregated G93A-SOD1 in transgenic mice with amyotrophic lateral sclerosis (ALS). We demonstrated that Alfy overexpression could decrease the expression of mutant proteins via the autophagosome-lysosome pathway, and thereby, the toxicity of mutant proteins was reduced. The clearance of the mutant proteins in NSC34 cells was significantly inhibited in an Alfy knockdown cellular model. We therefore deduced that Alfy translocalization likely is involved in the pathogenesis of ALS. Alfy may be developed into a useful target for ALS therapy. © 2014, The Society for In Vitro Biology.

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