• Title/Summary/Keyword: Ganglioside GQ1b

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Regulatory roles of ganglioside GQ1b in neuronal cell differentiation of mouse embryonic stem cells

  • Kwak, Dong-Hoon;Jin, Jung-Woo;Ryu, Jae-Sung;Ko, Kinram;Lee, So-Dam;Lee, Jeong-Woong;Kim, Ji-Su;Jung, Kyu-Yong;Ko, Ki-Sung;Ma, Jin-Yeul;Hwang, Kyung-A;Chang, Kyu-Tae;Choo, Young-Kug
    • BMB Reports
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    • v.44 no.12
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    • pp.799-804
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    • 2011
  • Gangliosides play an important role in neuronal differentiation processes. The regulation of ganglioside levels is related to the induction of neuronal cell differentiation. In this study, the ST8Sia5 gene was transfected into mESCs and then differentiated into neuronal cells. Interestingly, ST8Sia5 gene transfected mESCs expressed GQ1b by HPTLC and immunofluorescence analysis. To investigate the effects of GQ1b over-expression in neurogenesis, neuronal cells were differentiated from GQ1b expressing mESCs in the presence of retinoic acid. In GQ1b expressing mESCs, increased EBs formation was observed. After 4 days, EBs were co-localized with GQ1b and nestin, and GFAP. Moreover, GQ1b co-localized with MAP-2 expressing cells in GQ1b expressing mESCs in 7-day-old EBs. Furthermore, GQ1b expressing mESCs increased the ERK1/2 MAP kinase pathway. These results suggest that the ST8Sia5 gene increases ganglioside GQ1b and improves neuronal differentiation via the ERK1/2 MAP kinase pathway.

Overlap syndrome of Miller-Fisher syndrome/Pharyngeal-Cervical-Brachial variant-Guillain Barre Syndrome with anti-ganglioside complex antibodies

  • Lee, Suk-yoon;Oh, Seong-il;Huh, So-Young;Shin, Kyong Jin;Kim, Jong Kuk;Yoon, Byeol-A
    • Annals of Clinical Neurophysiology
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    • v.22 no.2
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    • pp.112-116
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    • 2020
  • Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) can present with overlapping features. A 56-year-old female developed ptosis and diplopia after an upper respiratory infection, and presented with facial palsy, dysarthria, brachial weakness, ataxia, and areflexia. Mild weakness of both legs appeared after a few days. Anti-ganglioside complex antibody were positive to IgG GM1/GQ1b and GQ1b/sulfatide antibodies. The present case suggests that the manifestation of overlap between MFS/PCB variants and GBS could be caused by antiganglioside complex antibodies.

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.

Differential Expression Patterns of Gangliosides in the Liver and Heart of NIH-miniature Pigs (NIH-미니돼지의 간과 심장에서 갱글리오시드의 서로 다른 발현 패턴)

  • Ryu, Jae-Sung;Chang, Kyu-Tae;Kim, Ji-Su;Kwak, Dong-Hoon;Lee, Young-Choon;Oh, Keon-Bong;Choo, Young-Kug
    • Journal of Life Science
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    • v.20 no.4
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    • pp.467-473
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    • 2010
  • Gangliosides are a major component of the plasma membrane of mammalian cells, which are directly involved in a variety of immunological events, including cell-to cell or cell-to-protein interactions. In this study, we investigated whether gangliosides, sialic acid-containing glycosphingolipids, are related to rejection during the xenotransplantation of NIH-miniature pig livers and hearts to humans. Both high performance thin-layer chromatography and immunohistochemistry analyses revealed that the expression of gangliosides in the liver tissue of NIH-miniature pigs was higher than that in the heart. Gangliosides GD3, GD1a, GD1b, GT1b and GQ1b were observed in both the liver and heart, whereas GQ1b was detected only in the liver, indicating that the ganglioside expression profiles are tissue specific. Moreover, other ganglio-series gangliosides, including GM3, were not detected in the livers and hearts of NIH-miniature pigs. Taken together, these results suggest that gangliosides may play important roles in immune responses in clinical xenotransplants of pig livers and hearts.