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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2018/03/25 19:49:36」(JST)

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1. クラッベ病 krabbe disease
2. ペリツェウス・メルツバッハー病 pelizaeus merzbacher disease
3. 成人における肺炎マイコプラズマ感染症 mycoplasma pneumoniae infection in adults
4. アスパルトアシラーゼ欠乏症（カナバン病） aspartoacylase deficiency canavan disease

English Journal

Phase separation of myelin sheath in Triton X-114 solution: predominant localizaion of the 21.5-kDa isoform of myelin basic protein in the lipid raft-associated domain.

Uruse M1, Yamamoto M, Sugawa M, Matsuura K, Sato Y, Seiwa C, Watanabe K, Aiso S, Asou H.Author information 1Center for Kampo Medicine, Keio University School of Medicine, 35 Shinano-machi, Shinjuku-ku, Tokyo 160-8582, Japan; Tsumura Research Laboratories, Tsumura & Co., Yoshiwara 3586, Ami-machi, Inashiki-gun, Ibaraki 300-1192, Japan; and Department of Anatomy, Keio University School of Medicine, 35 Shinano-machi, Shinjuku-ku, Tokyo 160-8582, Japan.AbstractMyelin basic protein (MBP) isoforms in the myelin sheath are known to have distinct intracellular expression patterns, which are profoundly related to functional specificity. Determining the differential localization of MBP isoforms is therefore important for understanding their pathophysiological roles. In this study, we have developed a new method for phase separation of myelin. The non-ionic detergent Triton X-114 is used to solubilize myelin sheath which then undergoes phase separation to yield four fractions. The lipid raft-associated proteins and lipids in each fraction were analysed by immunoblotting and lipid analysis, respectively. The present method gives two lipid raft-enriched fractions, one of them was found to contain only lipid raft-associated galactocerebroside and cholesterol as the major lipids. The 21.5-kDa MBP isoforms (21.5 MBP), both unphosphorylated and phosphorylated, were exclusively contained in this fraction. Phosphorylated 21.5 MBP (21.5 pMBP) has been shown to specifically disappear from demyelinated loci. The present analytical method clearly indicated that disappearance of 21.5 pMBP corresponded to demyelination and its reappearance corresponded to prevention of demyelination. Demyelination was also associated with aging and was prevented by the myelin-protecting herbal medicine, Chinpi, a type of dried citrus peel.
Journal of biochemistry.J Biochem.2014 Feb 18. [Epub ahead of print]
Myelin basic protein (MBP) isoforms in the myelin sheath are known to have distinct intracellular expression patterns, which are profoundly related to functional specificity. Determining the differential localization of MBP isoforms is therefore important for understanding their pathophysiological r
PMID 24459152

Clinical features in Guillain-Barré syndrome with anti-Gal-C antibody.

Samukawa M1, Hamada Y1, Kuwahara M1, Takada K1, Hirano M2, Mitsui Y1, Sonoo M3, Kusunoki S4; Japanese GBS Study Group.Author information 1Department of Neurology, Kinki University Faculty of Medicine, Osaka-Sayama, Japan.2Department of Neurology, Kinki University Faculty of Medicine, Osaka-Sayama, Japan; Department of Neurology, Sakai Hospital, Kinki University Faculty of Medicine, Sakai, Japan.3Department of Neurology, Teikyo University School of Medicine, Tokyo, Japan.4Department of Neurology, Kinki University Faculty of Medicine, Osaka-Sayama, Japan. Electronic address: kusunoki-tky@umin.ac.jp.AbstractINTRODUCTION: Guillain-Barré syndrome (GBS) has often been associated with antibodies to glycolipids, such as galactocerebroside (Gal-C), a component of myelin. Whether patients who have GBS with anti-Gal-C antibody (Gal-C-GBS) more often have demyelinating neuropathy or axonal neuropathy remains controversial. Their clinical features have also been unestablished.
Journal of the neurological sciences.J Neurol Sci.2014 Feb 15;337(1-2):55-60. doi: 10.1016/j.jns.2013.11.016. Epub 2013 Nov 19.
INTRODUCTION: Guillain-Barré syndrome (GBS) has often been associated with antibodies to glycolipids, such as galactocerebroside (Gal-C), a component of myelin. Whether patients who have GBS with anti-Gal-C antibody (Gal-C-GBS) more often have demyelinating neuropathy or axonal neuropathy remains c
PMID 24289889

The application of glycosphingolipid arrays to autoantibody detection in neuroimmunological disorders.

Galban-Horcajo F1, Halstead SK1, McGonigal R1, Willison HJ2.Author information 1Institute of Infection, Immunity and Inflammation, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow G12 8TA, United Kingdom.2Institute of Infection, Immunity and Inflammation, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow G12 8TA, United Kingdom. Electronic address: Hugh.Willison@glasgow.ac.uk.AbstractHumans with autoimmune peripheral neuropathies frequently harbour serum antibodies to single glycosphingolipids, especially gangliosides. Recently it has been appreciated that glycolipid and lipid complexes, formed from two or more individual species, can interact to create molecular shapes capable of being recognised by these autoantibodies whilst not binding to the single individuals. As a result of this, novel autoantibody targets have been identified. This newly termed 'combinatorial glycomic' approach has provided the impetus to redesigning the assay methodologies traditionally used in the neuropathy-associated autoantibody field. Combinatorial glycoarrays can be readily constructed in house using lipids of interest. Herein we especially highlight the role of the neutral lipids cholesterol and galactocerebroside in modifying glycosphingolipid orientation that subsequently favours or inhibits autoantibody binding.
Current opinion in chemical biology.Curr Opin Chem Biol.2014 Feb;18C:78-86. doi: 10.1016/j.cbpa.2014.01.008. Epub 2014 Feb 2.
Humans with autoimmune peripheral neuropathies frequently harbour serum antibodies to single glycosphingolipids, especially gangliosides. Recently it has been appreciated that glycolipid and lipid complexes, formed from two or more individual species, can interact to create molecular shapes capable
PMID 24495749