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1.
Article in Korean | WPRIM | ID: wpr-766753

ABSTRACT

Guillain-Barré syndrome (GBS) is a representative form of post-infectious autoimmune neuropathy with heterogenous manifestations. It was originally considered as an ascending demyelinating polyneuropathy in Western countries. However, the discovery of anti-ganglioside antibodies on the basis of molecular mimicry theory could help us better understand various kinds of focal and regional variants as well as axonal type of GBS those were frequently found from Asian countries. Recent development of new techniques about anti-ganglioside complex antibodies is making more detailed descriptions for specific or unusual clinical manifestations. It has been regarded that GBS has good prognosis if treated properly as early as possible, but it still shows high mortality and morbidity rate with frequent long term neurologic and medical complications. Unfortunately, there are only two options for medical treatment, intravenous immunoglobulin and plasmapheresis, for the last 100 years. Several clinical studies on new immunotherapy targeting complement activating system with background of molecular mimicry using animal model are underway. We hope that these new treatments will be helpful for the future patients.


Subject(s)
Humans , Antibodies , Asian People , Axons , Complement System Proteins , Gangliosides , Guillain-Barre Syndrome , Hope , Immunoglobulins , Immunotherapy , Miller Fisher Syndrome , Models, Animal , Molecular Mimicry , Mortality , Plasmapheresis , Polyneuropathies , Prognosis
2.
Journal of Neurocritical Care ; (2): 134-136, 2018.
Article in English | WPRIM | ID: wpr-765906

ABSTRACT

BACKGROUND: Recently, anti-ganglioside complex (GSC) antibodies were discovered among the various subtypes of Guillain-Barré syndrome. GSC is the novel glycoepitopes formed by two individual ganglioside molecules. CASE REPORT: We present a 36-year-old man with overlap Miller Fisher syndrome and acute bulbar palsy who had anti-GSC antibody that provided diagnostic robustness. CONCLUSION: Anti-GSC testing could be considered important in patients who show atypical manifestation with negative antibody reaction against each constituent ganglioside.


Subject(s)
Adult , Humans , Antibodies , Bulbar Palsy, Progressive , Gangliosides , Guillain-Barre Syndrome , Miller Fisher Syndrome
3.
Journal of Experimental Hematology ; (6): 1022-1026, 2018.
Article in Chinese | WPRIM | ID: wpr-689535

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the proliferation- inhibitory and apoptosis inducing effect of ganglioside GM3 on human multiple myeloma cell line U266 cells and its possible mechanisms.</p><p><b>METHODS</b>MTT assay and flow cytometry were used to observe the effects of GM3 ganglioside on proliferation and apoptosis of human myeloma cell line U266. Effects of different concentration of ganglioside GM3 on the mRNA expression level of BCL-2 and BAX were detected by Real-time PCR.</p><p><b>RESULTS</b>MTT assay and Flow Cytometry showed that ganglioside GM3 could induce the apoptosis and inhibit the proliferation of multiple myeloma U266 cell line, and both the effects were enhanced with the increase of GM3 ganglioside concentration. Compared with the control group, the relative expression of BAX mRNA with the increase of GM3 concentration in experimental group was enhanced gradually(r=0.968), while the relative mRNA expression of anti-apoptotic gene BCL-2 was decreased gradually(r=-0.727).</p><p><b>CONCLUSION</b>GM3 ganglioside can induce apoptosis and inhibit the proliferation of U266 cell line in a concentration dependent manner. The mechanism may be related with up- regulation of BAX expression and down-regulation of BCL-2.</p>


Subject(s)
Humans , Apoptosis , Cell Line, Tumor , Cell Proliferation , Gangliosides , Multiple Myeloma
4.
Conscientiae saúde (Impr.) ; 16(2): 2017289292, jun. 2017.
Article in Portuguese | LILACS | ID: biblio-875898

ABSTRACT

Introdução: A gangliosidose é uma doença caracterizada pelo acúmulo do substrato gangliosídeo nos lisossomos devido à deficiência da enzima betagalactosidase. É uma desordem rara, estimando-se uma incidência na população de 1:100.000 a 200.000. Clinicamente os pacientes apresentam graus variados de neurodegeneração e alterações esqueléticas, categorizadas pela gravidade e atividade residual da beta-galactosidase, podendo ocorrer dismorfismo facial, hepatoesplenomegalia, displasia esquelética, manchas maculares vermelhas, cegueira e até morte precoce. O atraso no desenvolvimento neuropsicomotor associada à degeneração do sistema nervoso central, pode levar o paciente a um quadro de hipotonia muscular generalizada progressiva, evoluindo para espasticidade e crises convulsivas. Objetivo: Relata-se caso de paciente masculino, apresentando alterações no desenvolvimento neuropsicomotor desde os oito meses, com elucidação diagnóstica através da clínica, exames de imagem e laboratoriais. Método: Busca em bancos de dados digitais artigos científicos que discorram sobre a gangliosidose. Resultados/Conclusão: A gangliosiodose é uma disordem rara, o que torna o relato de caso importante como fonte de pesquisa. (AU)


Introduction: Gangliosidosis is a disease characterized by accumulation of the ganglioside substrate in lysosomes due to beta-galactosidase enzyme deficiency. It is a rare disorder, with an incidence of 1: 100,000 to 200,000. Clinically the patients pres- ent varying degrees of neurodegeneration and skeletal changes, categorized by the gravity and residual activity of beta-galactosidase, being able to occur facial dysmorphism, hepatosplenomegaly, skeletal dysplasia, red macular spots, blind- ness and early death. Objective: Delayed neuropsychomotor development associated with degeneration of the central nervous system may lead the patient to progressive generalized muscular hypotonia, evolving into seizures and convulsive seizures. We report a case of male patient, presenting changes in neuropsychomotor devel- opment since eight months of age, with diagnostic elucidation through clinical exam, imaging and laboratory tests. Method: Search in digital databases for scientific articles that discuss gangliosidoses. Results/ conclusion: Gangliosidosis is rare disorder, which makes reporting an important source of research. (AU)


Subject(s)
Humans , Male , Infant , Gangliosidosis, GM1 , Child Development , Rare Diseases , Gangliosides
5.
Pakistan Journal of Medical Sciences. 2017; 33 (5): 1199-1204
in English | IMEMR | ID: emr-189775

ABSTRACT

Objective: To study the clinical effect of ganglioside [GM] and fructose-1, 6-diphosphate [FDP] on neonatal heart and brain injuries after asphyxia


Methods: Ninety-one neonates with asphyxia neonatal heart and brain injuries were randomly divided into an observation group and a control group. Both groups were given symptomatic treatment as soon as possible. On this basis, the observation group was given 200 ml of 5% glucose injection and 20 mg of GM and 250 mg/kg-d FDP by intravenous infusion. The above two drugs were given once a day for 14 days. The control group was given 20 ml of 5% glucose injection, 2 ml of cerebrolysin and 250 mg/kg-d FDP by intravenous infusion, once a day for 14 days. Both groups were administered on the first day after admission, and the course of treatment was 14 days. The treatment outcomes of the two groups were compared by detecting the levels of glycogen phosphorylase isoenzyme BB [GPBB], cTn-l and CK-MB, MRI results and Neonatal Behavioral Neurological Assessment [NBNA] scores before and after treatment


Results: The levels of GPBB, cTn-l and CK-MB in the observation group were significantly higher than those of normal neonates. After treatment, the levels of cTn-l and CK-MB in the observation group were closer to those of normal neonates compared with the control group, with significant differences [P<0.05]. There was a significant difference in the brain MRI examination between the two groups [P<0.05]. The NBNA scores of the two groups were significantly different before and after treatment [P<0.05]. The total effective rate of the observation group was significantly higher than that of the control group [P<0.05]. Conclusion: Neonatal heart and brain injuries after asphyxia can be well treated by combining GM with FDP


Subject(s)
Humans , Male , Female , Infant, Newborn , Fructosediphosphates , Heart Injuries , Brain Injuries , Asphyxia , Gangliosides , Infant, Newborn, Diseases , Creatine Kinase, MB Form/blood
6.
Article in Korean | WPRIM | ID: wpr-173343

ABSTRACT

Acute disseminated encephalomyelitis (ADEM) and Guillain-Barré syndrome (GBS) are both rare post-infectious neurological disorders. The co-existence of these conditions has often been reported despite of low incidence. We describe a 20-year-old male, who presented with acute flaccid paralysis and encephalopathy. The patient showed reversible MRI lesions suggesting ADEM. This case showed anti-GT1a IgG and anti-GM1 IgM antibodies positivity. We suggest that certain immunogenicity within central and peripheral nervous system may share a common autoimmune process during the disease course.


Subject(s)
Humans , Male , Young Adult , Antibodies , Brain Diseases , Encephalomyelitis, Acute Disseminated , Gangliosides , Guillain-Barre Syndrome , Immunoglobulin G , Immunoglobulin M , Incidence , Magnetic Resonance Imaging , Nervous System Diseases , Paralysis , Peripheral Nervous System
7.
Article in English | WPRIM | ID: wpr-203977

ABSTRACT

Our study aims to explore the effects of lentivirus-mediated microRNA-124 (miR-124) gene-modified bone marrow mesenchymal stem cell (BMSC) transplantation on the repair of spinal cord injury (SCI) in rats. BMSCs were isolated from the bone marrow of rats. The target gene miR-124 was identified using a luciferase-reporter gene assay. Seventy-two rats were selected for construction of the SCI model, and the rats were randomly divided into the blank group, sham group, SCI group, negative control (NC) group, overexpressed miR-124 group and si-PDXK group. The mRNA expression of miR-124 and the mRNA and protein expression of pyridoxal kinase (PDXK) were detected by quantitative real-time polymerase chain reaction and western blotting. The locomotor capacity of the rats was evaluated using the Basso, Beattie and Bresnahan (BBB) scale. Brdu, neuron-specific enolase (NSE), neurofilament (NF) and microtubule-associated protein 2 (MAP2) were detected using immunohistochemistry. The expression levels of thyrotropin-releasing hormone (TRH), prostacyclin (PGI2) and gangliosides (GM) were measured using an enzyme-linked immunosorbent assay. PDXK was identified as the target gene of miR-124. The overexpressed miR-124 group exhibited higher miR-124 expression than the SCI, NC and si-PDXK groups. Compared with the SCI and NC groups, the PDXK expression was downregulated in the overexpressed miR-124 and si-PDXK groups, and the BBB scores were significantly increased 7, 21 and 35 days after transplantation. The double-labeled positive cell densities (Brdu+NSE/NF/MAP2) and the expression levels of TRH, PGI2 and GM in the overexpressed miR-124 group were significantly higher than those in the NC and SCI groups. These results indicated that miR-124 targeted PDXK to accelerate the differentiation of BMSCs into neurocytes and promote SCI repair.


Subject(s)
Animals , Rats , Blotting, Western , Bone Marrow , Bromodeoxyuridine , Cell Count , Enzyme-Linked Immunosorbent Assay , Epoprostenol , Gangliosides , Immunohistochemistry , Intermediate Filaments , Mesenchymal Stem Cell Transplantation , Mesenchymal Stem Cells , Microtubule-Associated Proteins , Phosphopyruvate Hydratase , Pyridoxal Kinase , Real-Time Polymerase Chain Reaction , RNA, Messenger , Spinal Cord Injuries , Spinal Cord , Thyrotropin-Releasing Hormone
9.
Article in English | WPRIM | ID: wpr-100893

ABSTRACT

BACKGROUND/OBJECTIVES: We investigated the anti-osteoarthritic effects of deer bone extract on the gene expressions of matrix metalloproteinases (MMPs) and collagen type II (COL2) in interleukin-1β-induced osteoarthritis (OA) chondrocytes. MATERIALS/METHODS: Primary rabbit chondrocytes were treated as follows: CON (PBS treatment), NC (IL-1β treatment), PC (IL-1β + 100 µg/mL glucosamine sulphate/chondroitin sulphate mixture), and DB (IL-1β + 100 µg/mL deer bone extract). RESULTS: The results of the cell viability assay indicated that deer bone extract at doses ranging from 100 to 500 µg/mL inhibits cell death in chondrocytes induced by IL-1β. Deer bone extract was able to significantly recover the mRNA expression of COL2 that was down-regulated by IL-1β (NC: 0.79 vs. DB: 0.87, P < 0.05) and significantly decrease the mRNA expression of MMP-3 (NC: 2.24 vs. DB: 1.75) and -13 (NC: 1.28 vs. DB: 0.89) in OA chondrocytes (P < 0.05). CONCLUSIONS: We concluded that deer bone extract induces accumulation of COL2 through the down-regulation of MMPs in IL-1β-induced OA chondrocytes. Our results suggest that deer bone extract, which contains various components related to OA, including chondroitin sulphate, may possess anti-osteoarthritic properties and be of value in inhibiting the pathogenesis of OA.


Subject(s)
Cell Death , Cell Survival , Chondrocytes , Chondroitin , Collagen Type II , Collagen , Deer , Down-Regulation , Gangliosides , Gene Expression , Glucosamine , Matrix Metalloproteinases , Osteoarthritis , RNA, Messenger
10.
Article in English | WPRIM | ID: wpr-96073

ABSTRACT

BACKGROUND AND PURPOSE: Abnormalities of the peripheral nervous system occur in 5% of patients with lymphoma. Polyneuropathy has not been described in patients with mantle-cell and marginal-zone B-cell lymphomas. CASE REPORT: Two elderly patients with indolent non-Hodgkin's lymphoma developed a progressive sensory polyneuropathy that was associated with serum autoantibodies directed against asialosyl/sialosyl gangliosides and myelin-associated glycoprotein/sulfated glucuronyl paragloboside, respectively, which are peripheral-nerve antigens. The oligoclonal pattern of these antibodies hinted at a lymphoma-induced immune dysregulation. The neuropathy stabilized clinically during treatment with intravenous immunoglobulin G. B-cell lymphoma was managed with a "watchful waiting" approach. CONCLUSIONS: The concept of antigen-specific, immune-mediated neuropathy associated with slow-growing lymphoma of mature B-cells may be underrecognized. The principle of treating the illness underlying neuropathy may not be always indicated or necessary if risk-benefit and cost-benefit analyses are taken into account.


Subject(s)
Aged , Humans , Antibodies , Autoantibodies , Autoimmunity , B-Lymphocytes , Cost-Benefit Analysis , Gangliosides , Immunoglobulin G , Lymphoma , Lymphoma, B-Cell , Lymphoma, Non-Hodgkin , Peripheral Nervous System , Polyneuropathies
11.
National Journal of Andrology ; (12): 447-457, 2015.
Article in Chinese | WPRIM | ID: wpr-276076

ABSTRACT

<p><b>OBJECTIVE</b>To evaluate the clinical efficacy and safety of phosphodiesterase 5 (PDE-5) inhibitors for erectile dysfunction (ED) in patients with diabetes mellitus and provide some evidence for the clinical treatment of the disease.</p><p><b>METHODS</b>We searched MedMed, EMbase, Cochrane Library, CNKI, Wan Fang Data, VIP and ZADL for randomized controlled trials on PDE-5 inhibitors for ED in diabetic men and evaluated the methodology of the included trials with the Jadad scale. We used the erectile function domain in the IIEF (IIEF-EF), IIEF questions (IIEF-Q) 3 and 4, SEP-2 and -3, and Global Assessment Questions (GAQ) as the main evaluation indexes and employed the Review Manager 5. 1. 0 software for meta analysis.</p><p><b>RESULTS</b>A total of 13 studies were included, which were all high quality trials with Jadad score > 3. The IIEF-EF scores in 10 of the included studies were subjected to meta analysis using the random-effect model (REM), with a weighted mean difference (WMD) of 5.64 (95% CI 4.41 - 6.83, P < 0.001). The fixed-effect model (FEM) analysis of the IIEF-Q scores in 6 of the studies showed the WMD to be 0.96 (95% CI 0.83 -1.08, P < 0.001) for IIEF-Q3 and 1.11 (95% CI 0.98 - 1.25, P < 0.001) for IIEF-Q4. FEM analysis of the SEP-2 scores showed WMD = 17.67 (95% CI 12. 38 - 22. 97, P < 0.001) in 2 of the studies, and that of the SEP-3 scores WMD = 23.64 (95% CI 17. 49 - 29.79, P < 0.001) in 5 of the studies. The GAQ scores in 11 of the studies were subjected to REM analysis, with OR = 6. 20 and 95% CI 3.65 - 10.52 (P < 0.001). REM analysis was performed on the adverse reactions in 11 of the studies, with OR = 7.43 and 95% CI 4.11 - 13.44 (P < 0.001).</p><p><b>CONCLUSION</b>PDE-5 inhibitors can effectively and safely improve erectile function in patients with diabetes mellitus.</p>


Subject(s)
Humans , Male , Diabetes Mellitus , Erectile Dysfunction , Drug Therapy , Gangliosides , Penile Erection , Phosphodiesterase 5 Inhibitors , Therapeutic Uses
12.
Article in Chinese | WPRIM | ID: wpr-312804

ABSTRACT

<p><b>OBJECTIVE</b>To observe the efficacy of integrative medical sequential method in treating cerebral palsy (CP) children's intelligence development, muscular tension, serum interleukin 6 (IL-6), and tumor necrosis factor alpha (TNF-alpha).</p><p><b>METHODS</b>Totally 111 CP children were randomly assigned to the control group (50 cases) and the treatment group (61 cases). All patients received comprehensive rehabilitation training and intravenous dripping of Monosialotetrahexosylganglioside Sodium Injection for 10 days. But those in the treatment group additionally received Chinese medical enema for brain resuscitation, relieving rigidity of muscles and activating collaterals for 14 days. Then they started another medication cycle and lasted for a total of 6 cycles. Serum IL-6 levels and TNF-alpha contents were determined before treatment. Scoring for muscular tension, Gesell score for intelligence development, contents of serum IL-6 and TNF-alpha were assessed before and after treatment in the two groups.</p><p><b>RESULTS</b>Compared with before treatment in this group, muscular tension, Gesell scores for intelligence development all decreased in the two groups (P < 0.05). As for inter-group comparison, the decrement was more obvious in the treatment group than in the control group (P < 0.05). The total effective rate was 86.9% in the treatment group and 76.0% in the control group (P < 0.05). The contents of IL-6 and TNF-alpha were obviously reduced in the treatment group and the control group after treatment (P < 0.01). The decrement was more obvious in the treatment group (P < 0.05).</p><p><b>CONCLUSION</b>The two treatment methods were effective for CP children, but the efficacy was superior in the treatment group than in the control group, indicating integrative medical methods could play a synergistic effect and optimize the treatment program for CP.</p>


Subject(s)
Child, Preschool , Female , Humans , Infant , Male , Cerebral Palsy , Drug Therapy , Drugs, Chinese Herbal , Therapeutic Uses , Gangliosides , Therapeutic Uses , Integrative Medicine , Intelligence , Interleukin-6 , Blood , Phytotherapy , Tumor Necrosis Factor-alpha , Blood
13.
Rev. méd. Chile ; 141(9): 1211-1215, set. 2013. tab
Article in Spanish | LILACS | ID: lil-699689

ABSTRACT

Anti-GQ1b syndrome includes Miller Fisher Syndrome (MFS), Guillain Barré Syndrome (GBS), Bickerstaff`s brain stem encephalitis (BBE) and Acute Ophtamoplegia (AO). We report four patients aged 16 to 76 years, with anti-GQ1b syndrome. All presented with MFS, one of them evolved to GBS pharyngeal-cervical-brachial variant and other to GBS with BBE. All had a previous history of diarrhea or upper respiratory tract infection. All had positive anti-GQ1b serum antibodies. Both brain magnetic resonance imaging and cerebrospinal fluid analysis were normal. Electrophysiology studies were compatible with a demyelinating disease. Two patients needed airway protection with an orotracheal tube and developed dysautonomia. All four patients were treated with immunomodulation. On the sixth month follow-up, patients had only minimal alterations in the neurological examination.


Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Antibodies, Anti-Idiotypic/blood , Encephalitis/diagnosis , Gangliosides/blood , Guillain-Barre Syndrome/diagnosis , Miller Fisher Syndrome/diagnosis , Ophthalmoplegia/diagnosis , Brain Stem , Encephalitis/drug therapy , Gangliosides/immunology , Guillain-Barre Syndrome/drug therapy , Immunoglobulins, Intravenous/therapeutic use , Magnetic Resonance Imaging , Miller Fisher Syndrome/drug therapy , Ophthalmoplegia/drug therapy
14.
Article in English | WPRIM | ID: wpr-86388

ABSTRACT

Reported herein is an adult case of Fisher syndrome (FS) that occurred as a complication during the course of community-acquired pneumonia caused by Mycoplasma pneumoniae. A 38-yr-old man who had been treated with antibiotics for serologically proven M. pneumoniae pneumonia presented with a sudden onset of diplopia, ataxic gait, and areflexia. A thorough evaluation including brain imaging, cerebrospinal fluid examination, a nerve conduction study, and detection of serum anti-ganglioside GQ1b antibody titers led to the diagnosis of FS. Antibiotic treatment of the underlying M. pneumoniae pneumonia was maintained without additional immunomodulatory agents. A complete and spontaneous resolution of neurologic abnormalities was observed within 1 month, accompanied by resolution of lung lesions.


Subject(s)
Adult , Humans , Male , Anti-Bacterial Agents/therapeutic use , Antibodies/blood , Diplopia/etiology , Erythrocyte Count , Gangliosides/immunology , Lung/diagnostic imaging , Miller Fisher Syndrome/diagnosis , Pneumonia, Mycoplasma/complications , Tomography, X-Ray Computed
17.
Article in Chinese | WPRIM | ID: wpr-749409

ABSTRACT

OBJECTIVE@#To evaluate the efficiency between two treatments of sudden hearing loss.@*METHOD@#All patients were divided into two groups randomly, basic drug group was treated with ganglioside and vinpocetine injection, combined therapy group was treated with intratympanic dexamethasone and what was used in basic drug group.@*RESULT@#The effective rate of combined therapy group (73.53%) was significantly higher than that of basic drug group (37.78%) (P < 0.05).@*CONCLUSION@#The comprehensive therapy of intratympanic dexamethasone injection, ganglioside and vinpocetine injection have excellent efficiency for sudden hearing loss.


Subject(s)
Adolescent , Adult , Child , Female , Humans , Male , Middle Aged , Young Adult , Dexamethasone , Therapeutic Uses , Drug Therapy, Combination , Ear, Middle , Gangliosides , Therapeutic Uses , Hearing Loss, Sudden , Drug Therapy , Injections , Treatment Outcome , Vinca Alkaloids , Therapeutic Uses
18.
Laboratory Animal Research ; : 255-263, 2012.
Article in English | WPRIM | ID: wpr-192523

ABSTRACT

Gangliosides are ubiquitous components of the membranes of mammalian cells that are thought to play important roles in various cell functions such as cell-cell interaction, cell adhesion, cell differentiation, growth control, and signaling. However, the role that gangliosides play in the immune rejection response after xenotransplantation is not yet clearly understood. In this study, the regulatory effects of human leukocytes on ganglioside expression in primary cultured micro-pig aortic endothelial cells (PAECs) were investigated. To determine the impact of human leukocytes on the expression of gangliosides in PAECs, we performed high-performance thin layer chromatography (HPTLC) in PAECs incubated with FBS, FBS containing human leukocytes, human serum containing human leukocytes, and FBS containing TNF-alpha. Both HPTLC and immunohistochemistry analyses revealed that PAECs incubated with FBS predominantly express the gangliosides GM3, GM1, and GD3. However, the expression of GM1 significantly decreased in PAECs incubated for 5 h with TNF-alpha (10 ng/mL), 10% human serum containing human leukocytes, and 10% FBS containing human leukocytes. Taken together, these results suggest that human leukocytes induced changes in the expression profile of ganglioside GM1 similar to those seen upon treatment of PAECs with TNF-alpha. This finding may be relevant for designing future therapeutic strategies intended to prolong xenograft survival.


Subject(s)
Humans , Cell Adhesion , Cell Communication , Chromatography, Thin Layer , Endothelial Cells , Gangliosides , Immunohistochemistry , Leukocytes , Membranes , Rejection, Psychology , Transplantation, Heterologous , Tumor Necrosis Factor-alpha
19.
Article in Korean | WPRIM | ID: wpr-30458

ABSTRACT

PURPOSE: To investigate the role of gangliosides in the differentiation of orbital fibroblasts into adipocytes, a component in the pathogenesis of Graves' ophthalmopathy. METHODS: Orbital tissues were obtained during orbital surgery for subjects without Graves' ophthalmopathy or other inflammatory orbital disease, and orbital fibroblasts were primarily cultured from each obtained tissue. Morphological examination of orbital fibroblasts was performed after treatment with commercially available gangliosides mixture (Gmix) comprised of several subtypes. To determine the effect of Gmix on the differentiation of orbital fibroblasts into adipocytes and the differentiation-related genes, Oil Red-O staining and RT-PCR were performed. RESULTS: The treatment with Gmix induced the morphological changes, which at least in part were explained with the differentiation of orbital fibroblasts into adipocytes in accordance with the increase of mRNA level of genes known to be related to adipogenesis, whereas dermal fibroblasts and preadipocytes were irresponsive to the same treatment. CONCLUSIONS: The results from the present study suggest gangliosides may have a role in pathologic mechanisms of Graves' ophthalmopathy by the induction of differentiation of orbital fibroblasts into adipocytes.


Subject(s)
Adipocytes , Adipogenesis , Fibroblasts , Gangliosides , Orbit , Orbital Diseases , RNA, Messenger
20.
Article in Korean | WPRIM | ID: wpr-101463

ABSTRACT

Gangliosides are components of the membranous constituents and abundant in the nervous system. And they are implicated in a wide range of biological activities including the regulation of cell proliferation, differentiation and lysosomal activity. But they have a diverse action to induce neuronal cell death by the interaction with some ligands. The interference of their biosynthesis is accompanied by the intracellular accumulation of unwanted and neurotoxic proteins and might underlie the neurodegeneration diseases including Parkinson's disease. However the mechanism has not been elucidated. In this study, we report that the enhancement of biosynthesis of ganglioside GD3 protects the intracellular accumulation of alpha-synuclein and neuronal death. PC12 cells, dopaminergic neurons are cultured with synthetic proteasomal inhibitor (PSI, Z-lle-Glu(OtBu)-Ala-Leu-al) and L-PDMP (GD3 synthetase enhancer, L-threo-1-phenyl-2-decanoylamino-3-morpholino-1-propanol). We found that the neuronal viability was recovered by L-PDMP from the proteasomal inhibition and also the expression of activated caspase-3 and PARP was reduced. L-PDMP decreased in the intracellular accumulation of alpha-synuclein. Interestingly, PSI induced the expression of ganglioside3 in PC12 cell. Our findings suggest that proteasomal inhibition may modulate the biosynthesis of GD3 and L-PDMP protects dopaminergic neurons from death by proteasomal inhibition and the accumulation of alpha-synuclein.


Subject(s)
Animals , alpha-Synuclein , Caspase 3 , Cell Death , Cell Proliferation , Dopaminergic Neurons , Gangliosides , Ligands , Ligases , Nervous System , Neurons , Parkinson Disease , PC12 Cells , Proteins
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