• Title/Summary/Keyword: Ganglioside

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GLB1-related disorders: GM1 gangliosidosis and Morquio B disease

  • Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of Genetic Medicine
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    • v.18 no.1
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    • pp.16-23
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    • 2021
  • GLB1-related disorders comprise two phenotypically unique disorders: GM1 gangliosidosis and Morquio B disease. These autosomal recessive disorders are caused by b-galactosidase deficiency. A hallmark of GM1 gangliosidosis is central nervous system degeneration where ganglioside synthesis is highest. The accumulation of keratan sulfate is the suspected cause of the bone findings in Morquio B disease. GM1 gangliosidosis is clinically characterized by a neurodegenerative disorder associated with dysostosis multiplex, while Morquio B disease is characterized by severe skeletal manifestations and the preservation of intelligence. Morquio B disease and GM1 gangliosidosis may be on a continuum of skeletal involvement. There is currently no effective treatment for GLB1-related disorders. Recently, multiple interventions have been developed and there are several ongoing clinical trials.

Isolated facial diplegia variant of Guillain-Barré syndrome with anti-GM1 IgG antibody

  • Jung, Jin Ho;Lee, Sukyoon;Seo, Jung Hwa;Bae, Jong Seok;Shin, Kyong Jin;Kim, Jong Kuk;Yoon, Byeol-A;Oh, Seong-il
    • Annals of Clinical Neurophysiology
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    • v.24 no.1
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    • pp.17-20
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    • 2022
  • Facial diplegia (FD) rarely occurs as a regional Guillain-Barré syndrome (GBS) variant. A 70-year-old male presented with bifacial weakness that had started on the left side and extended to the right after several days. He was then treated using steroids and gradually improved. Serum antiganglioside antibody testing revealed positivity for anti-GM1 IgG antibodies. FD can be idiopathic, but it is an uncommon GBS variant. The ganglioside antibody test may increase the possibility of diagnosing isolated FD.

Bilateral Sixth Nerve Palsies as the Sole Manifestation of Atypical Miller-Fisher Syndrome (양측 외전신경마비만을 보인 비전형적 Miller-Fisher 증후군)

  • Nho, Sang-Woo;Kim, Jong-Kuk;Park, Whan-Seok;Jung, Eun-Joo;Kim, Sang-Jin;Kim, Eung-Gyu;Bae, Jong-Seok
    • Annals of Clinical Neurophysiology
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    • v.13 no.1
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    • pp.51-53
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    • 2011
  • Recently, we encountered a man with isolated bilateral sixth nerve palsies and areflexia whose titer of anti-GQ1b IgG antibody was elevated. We propose that bilateral sixth nerve palsies can be the sole manifestation of "anti-GQ1b antibody syndromes" and that patients with isolated bilateral sixth nerve palsies should be administered an anti-GQ1b antibody test for the diagnosis of acute immune-related neuropathy.

Expression of Cholera Toxin B Subunit and Assembly as Functional Oligomers in Silkworm

  • Gong, Zhao-Hui;Jin, Hui-Qing;Jin, Yong-Feng;Zhang, Yao-Zhou
    • BMB Reports
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    • v.38 no.6
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    • pp.717-724
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    • 2005
  • The nontoxic B subunit of cholera toxin (CTB) can significantly increase the ability of proteins to induce immunological tolerance after oral administration, when it was conjugated to various proteins. Recombinant CTB offers great potential for treatment of autoimmune disease. Here we firstly investigated the feasibility of silkworm baculovirus expression vector system for the cost-effective production of CTB under the control of a strong polyhedrin promoter. Higher expression was achieved via introducing the partial non-coding and coding sequences (ATAAAT and ATGCCGAAT) of polyhedrin to the 5' end of the native CTB gene, with the maximal accumulation being approximately 54.4 mg/L of hemolymph. The silkworm bioreactor produced this protein vaccine as the glycoslated pentameric form, which retained the GM1-ganglioside binding affinity and the native antigenicity of CTB. Further studies revealed that mixing with silkworm-derived CTB increases the tolerogenic potential of insulin. In the nonconjugated form, an insulin : CTB ratio of 100 : 1 was optimal for the prominent reduction in pancreatic islet inflammation. The data presented here demonstrate that the silkworm bioreactor is an ideal production and delivery system for an oral protein vaccine designed to develop immunological tolerance against autoimmune diabetes and CTB functions as an effective mucosal adjuvant for oral tolerance induction.

Studies on the Analysis of Constituents of Deer Horn(II) -Analysis of gangliosides and free amino acids- (산지별 녹용(鹿茸)류의 성분분석 연구(II) -Ganglioside 및 유리 아미노산의 분석-)

  • Hong, Nam-Doo;Won, Do-Hee;Kim, Nam-Jae;Chang, Seung-Youb;Youn, Whang-Geum;Kim, Hae-Soo
    • Korean Journal of Pharmacognosy
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    • v.24 no.1
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    • pp.38-46
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    • 1993
  • Two kinds of gangliosides contained in deer horns were determined by integrating the peaks of TLC densitometry. Japanese deer horn originated from China showed the highest gangliosides among tested samples and the upper parts in deer horns showed higher gangliosides than the lower parts. In the case of graded samples, the best grade A showed the highest content and the worst grade E did the lowest content. Sixteen kinds of free amino acids were analyzed by auto amino acid analyzer. The lower region the deer horn was, the more the total content of free amino acids was and several kinds of amino acids were contained quite regularly.

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Differential Expression Patterns of Gangliosides in the Ischemic Cerebral Cortex Produced by Middle Cerebral Artery Occlusion

  • Kwak, Dong Hoon;Kim, Sung Min;Lee, Dea Hoon;Kim, Ji Su;Kim, Sun Mi;Lee, Seo Ul;Jung, Kyu Yong;Seo, Byoung Boo;Choo, Young Kug
    • Molecules and Cells
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    • v.20 no.3
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    • pp.354-360
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    • 2005
  • Neuronal damage subsequent to transient cerebral ischemia is a multifactorial process involving several overlapping mechanisms. Gangliosides, sialic acid-conjugated glycosphingolipids, reduce the severity of acute brain damage in vitro. However their in vivo effects on the cerebral cortex damaged by ischemic infarct are unknown. To assess the possible protective role of gangliosides we examined their expression in the cerebral cortex damaged by ischemic infarct in the rat. Ischemia was induced by middle cerebral artery (MCA) occlusion, and the resulting damage was observed by staining with 2, 3, 5-triphenylterazolium chloride (TTC). High-performance thin-layer chromatography (HPTLC) showed that gangliosides GM3 and GM1 increased in the damaged cerebral cortex, and immunofluorescence microscopy also revealed a significant change in expression of GM1. In addition, in situ hybridization demonstrated an increase in the mRNA for ganglioside GM3 synthase. These results suggest that gangliosides GM1 and GM3 may be synthesized in vivo to protect the cerebral cortex from ischemic damage.

Tertiary Structure of Ganglioside $G_{A1}$ as Determined by NMR Spectroscopy

  • 이경익;이상원;전길자;김양미
    • Bulletin of the Korean Chemical Society
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    • v.19 no.5
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    • pp.569-575
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    • 1998
  • Investigation of the structure of the gangliosides has proven to be very important in the understanding of their biological roles. We have determined the tertiary structure of asialoganglioside GM1 $(GA_1)$ using NMR spectroscopy and distance geometry calculations. All of the structures are very similar except the glycosidic torsion angles in the ring IV and ring III linkages. There are two low-energy structures for GA1, G1 and G2. G1 differs from G2 only in the IV-III glycosidic linkages and the orientation of acetamido group in ring III. There is a stable intramolecular hydrogen bond between the third hydroxyl group in ring I and the ring oxygen atom in ring II. Also, there may be a weak hydrogen bond between the second hydroxyl group in ring IV and the acetamido group in ring III. Small coupling constants of $^3J_{IH3,IOH3}\; and\; ^3J_{IVH2,IVOH2}$ support this result. Overall structural features of $(GA_1)$ are very similar to those of $(GM_1)$. It implicates that specificities of the sugar moieties in GM1 are caused not by their tertiary foldings, but mainly by the electrostatic interactions between the polar sialic acid and its receptors. Since it is evident that $(GA_1)$ is more hydrophobic than $(GA_1)$, a receptor with a hydrophobic binding site can recognize the $(GA_1)$ better than $(GA_1)$. Studies on the conformational properties of $(GA_1)$ may lead to a better understanding of the molecular basis of its functions.

A Literature Study on Acupuncture for Cerebral Palsy -Based on the Current Traditional Chinese Medical Journals- (뇌성마비의 침자(鍼刺) 치료에 대한 문헌적 고찰 -최근 중의잡지를 중심으로-)

  • Ha, Su-Yun;Min, Sang-Yeon;Kim, Jang-Hyun
    • The Journal of Pediatrics of Korean Medicine
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    • v.23 no.1
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    • pp.205-228
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    • 2009
  • Objectives The purpose of this study is to investigate the current acupuncture therapy of cerebral palsy. Methods We investigated the Chinese clinical papers which were published in the last 10 years(from 1999 to 2008). We found these papers from the oriental medical library in university and we also used the China National Knowledge Infrastructure(CNKI) through the internet and selected 32 papers for analyzing. Results Most papers were described the effect of acupuncture or acupoint-injection. This is more effective way to treat than the general rehabilitation treatment such as the physical therapy, the occupational therapy, and the speech therapy. Acupuncture or acupoint-injection has overall $80{\sim}100%$ of rehabilitation rate. The younger the children were, the longer the treatment period was, and the more successful in treatment. The acupuncture was often used with the general acupuncture and scalp acupuncture. Commonly used major acupuncture points were sishencong(四神聰), bohui(百會), zusanli(足三里), yundongqu(運動區), pinghengqu(平衡區), quchi(曲池), and sanyinjiao(三陰交). Commonly used main meridian pathways were bladder, governor vessel, gallbladder, large intestine, stomach, small intestine meridian. Head is the common site for acupuncture. The main acupoint-injection points were zusanli(足三里), dazhui(大椎), shenshu(腎兪), yamen(啞門), neiguan(內關), and fengchi(風池). For the injection, brain activator, ganglioside M1, cerebroprotein hydrolysate, cytidine diphosphate choline, Vit B1, Vit B12, the salviae root, the safflower were commonly used. Conclusions Acupuncture and acupoint-injection have been shown as an effective treatment on cerebral palsy. The acupuncture was used often the general acupuncture and scalp acupuncture all together. Commonly used main acupuncture points were sishencong, bohui, zusanli, yundongqu, pinghengqu, quchi, sanyinjiao.

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Diagnosis Value of Membrane Glycolipids Biochemistry Index in Intracranial and Gastrointestinal Tumors

  • Lv, Jun;Lv, Can-Qun;Mei, Ping;Qi, Shi-Mei
    • Asian Pacific Journal of Cancer Prevention
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    • v.16 no.7
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    • pp.2693-2696
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    • 2015
  • The diagnostic value of membrane glycolipid biochemistry index, the lipid-bound sialic acid (LSA) and total sialic acid (TSA) in cerebrospinal fluid (CSF) was evaluated in 30 intracranial and 65 gastrointestinal tumors. The plasma LSA, TSA and red cell membrane sialic acid (R-SA) in were determined according to the method of Sevenmerhulm. Our results showed that the levels of LSA and TSA in CSF of intracranial tumor patients was higher than that of normal group(p<0.01). The concentration of TSA and LSA in patients with malignant glioma was higher than that of benign meningioma patients(P<0.01). No significance was found between intracranial halmatoma patients and normal control group for levels of membrane glycolipids (p>0.05). Results also found that the plasma LSA, TSA and R-SA of gastric carcinoma were significantly higher than those of control group (p<0.05); while no significant difference was found in the plasma LSA, TSA and R-SA levels between chronic gastritis, gastrohelcoma and normal control group (p>0.05). Plasma LSA, TSA and R-SA levels of gastric carcinoma patient were significantly higher than those of chronic gastritis patients and gastrohelcoma patients(p<0.05). It was also found that plasma LSA, TSA and R-SA contents were significantly higher in large intestine carcinoma patients than in benign in stestine tumor patients (p<0.05) while no significant difference was found between intestine benign tumor and normal control group (p>0.05). The levels of LSA, TSA and R-SA were obviously higher in the patients with metastasis than in the ones without (p<0.05.) The membrane glycolipid biochemistry index LSA and TSA in CSF are sensive markers for diagnosing intracranial tumors. For gastrointestinal malignant tumors the plasma LSA TSA and red blood cell membrane SA may be considered as auxiliary indicators for diagnosis. They can be used for distinguishing benign from malignant tumors.

Effects of IL-3 and SCF on Histamine Production Kinetics and Cell Phenotype in Rat Bone Marrow-derived Mast Cells

  • Lee, Haneul Nari;Kim, Chul Hwan;Song, Gwan Gyu;Cho, Sung-Weon
    • IMMUNE NETWORK
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    • v.10 no.1
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    • pp.15-25
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    • 2010
  • Background: Rat mast cells were regarded as a good model for mast cell function in immune response. Methods: Rat bone marrow mast cells (BMMC) were prepared both by recombinant rat IL-3 (rrIL-3) and by recombinant mouse stem cell factor (rmSCF), and investigated for both proliferation and differentiation in time course. Rat BMMC was induced by culture of rat bone marrow cells (BMCs) in the presence of both rrIL-3 (5 ng/ml) and rmSCF (5 ng/ml). Culture media were changed 2 times per week with the cell number condition of $5{\times}10^4/ml$ in 6 well plate. Proliferation was analyzed by cell number and cell counting kit-8 (CCK-8) and differentiation was by rat mast cell protease (RMCP) II and histamine. Results: Cell proliferation rates reached a maximum at 8 or 11 days of culture and decreased thereafter. However, both RMCP II production and histamine synthesis peaked after 11 days of culture. By real time RT-PCR, the level of histidine decarboxylase mRNA was more than 500 times higher on culture day 11 than on culture day 5. By transmission electron microscopy, the cells were heterogeneous in size and contained cytoplasmic granules. Using gated flow cytometry, we showed that cultured BMCs expressed high levels of $Fc{\varepsilon}RI$ and the mast cell antigen, ganglioside, on culture day 11. Conclusion: These results indicate that rat BMMCs were generated by culturing BMCs in the presence of rrII-3 and rmSCF and that the BMMCs have the characteristics of mucosal mast cells.