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Among hereditary disorders with membrane lipid abnormalities, a marked increase of free cholesterol (FC) and of phosphatidyl choline (PC) was detected in the red cells of 7 cases of hereditary high red cell membrane PC hemolytic anemia (HPCHA) and one case of congenital lecithin : cholesterol acyltransferase (LCAT) deficiency. Na+ influx was elevated significantly in the HPCHA red cells, contrary to the decreased Na+ influx in LCAT deficiency. In 4 cases of congenital β-lipoprotein (β-LP) deficiency with a marked decrease of plasma lipids, total membrane lipids were maintained almost normally, even though a moderate decrement of PC and an increased sphingomyelin were observed. In the disorder, Ca2+ uptake was markedly increased (approximately 100 fold greater than those in normal control), with normal Na+ influx. Among red cell membrane protein abnormalities, moderate decrement of FC, total phospholipids (PL), and phosphatidyl ethanolamine in the non-splenectomized hereditary spherocytosis patients was normalized after splenectomy. Increased Na+ influx, however, was not improved after the treatment, suggesting a possible contribution of the abnormal membrane proteins to increased Na+ influx, if they may exist. In hereditary stomatocytosis, various membrane lipid abnormalities were observed dependent on the type of the disorders. To clarify a possible role of membrane lipids on the abnormal membrane transport functions, such as Na+ influx, normal red cells were treated with phospholipase A2 (PLase A2) to convert PC to lyso-PC (L-PC). In some experiments, the PLase A2-treated red cells were further incubated with albumin to cleave membrane L-PC, which was formed by the enzymatic degradation of membrane PC. In the PLase A2-treated red cells, Na+ influx and Ca2+ uptake were markedly elevated. Ca2+ uptake increases further in the presence of albumin, in contrast to no response in Na+ influx. In conclusion, (1) membrane lipid abnormalities do affect the membrane transport functions, and (2) a different mechanism would exist between Na+ influx and Ca2+ uptake."}]}, "item_creator": {"attribute_name": "著者", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "橋本, 正志", "creatorNameLang": "ja"}, {"creatorName": "ハシモト, マサシ", "creatorNameLang": "ja-Kana"}], "nameIdentifiers": [{"nameIdentifier": "119882", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Hashimoto, Masashi", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "119883", "nameIdentifierScheme": "WEKO"}]}]}, "item_files": {"attribute_name": "ファイル情報", "attribute_type": "file", "attribute_value_mlt": [{"accessrole": "open_date", "date": [{"dateType": "Available", "dateValue": "2017-01-23"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "KJ00009816279.pdf", "filesize": [{"value": "1.6 MB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_note", "mimetype": "application/pdf", "size": 1600000.0, "url": {"label": "KJ00009816279.pdf", "objectType": "fulltext", "url": "https://kwmed.repo.nii.ac.jp/record/591/files/KJ00009816279.pdf"}, "version_id": "9e27ab0d-49a2-4abe-9506-427ecaddc459"}]}, "item_keyword": {"attribute_name": "キーワード", "attribute_value_mlt": [{"subitem_subject": "Membrane lipid abnormalities", "subitem_subject_language": "en", "subitem_subject_scheme": "Other"}, {"subitem_subject": "Membrane transport", "subitem_subject_language": "en", "subitem_subject_scheme": "Other"}, {"subitem_subject": "Phospholipase A2", "subitem_subject_language": "en", "subitem_subject_scheme": "Other"}]}, "item_language": {"attribute_name": "言語", "attribute_value_mlt": [{"subitem_language": "jpn"}]}, "item_resource_type": {"attribute_name": "資源タイプ", "attribute_value_mlt": [{"resourcetype": "journal article", "resourceuri": "http://purl.org/coar/resource_type/c_6501"}]}, "item_title": "赤血球膜脂質異常症の研究 : 膜脂質異常と膜輸送能との代謝的相関に関する臨床的および実験的研究", "item_titles": {"attribute_name": "タイトル", "attribute_value_mlt": [{"subitem_title": "赤血球膜脂質異常症の研究 : 膜脂質異常と膜輸送能との代謝的相関に関する臨床的および実験的研究", "subitem_title_language": "ja"}, {"subitem_title": "Studies of Erythrocyte Membrane Lipid Abnormalities : Clinical and Experimental Studies to Elucidate the Relationship between Membrane Lipid Abnormality and Membrane Transport", "subitem_title_language": "en"}]}, "item_type_id": "3", "owner": "25", "path": ["429"], "permalink_uri": "https://kwmed.repo.nii.ac.jp/records/591", "pubdate": {"attribute_name": "PubDate", "attribute_value": "2017-01-23"}, "publish_date": "2017-01-23", "publish_status": "0", "recid": "591", "relation": {}, "relation_version_is_last": true, "title": ["赤血球膜脂質異常症の研究 : 膜脂質異常と膜輸送能との代謝的相関に関する臨床的および実験的研究"], "weko_shared_id": -1}
赤血球膜脂質異常症の研究 : 膜脂質異常と膜輸送能との代謝的相関に関する臨床的および実験的研究
https://kwmed.repo.nii.ac.jp/records/591
https://kwmed.repo.nii.ac.jp/records/591a3068017-33ea-4c38-ab9d-2c5be5ae6b05
名前 / ファイル | ライセンス | アクション |
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KJ00009816279.pdf (1.6 MB)
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Item type | [ELS]学術雑誌論文 / Journal Article(1) | |||||
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公開日 | 2017-01-23 | |||||
タイトル | ||||||
言語 | ja | |||||
タイトル | 赤血球膜脂質異常症の研究 : 膜脂質異常と膜輸送能との代謝的相関に関する臨床的および実験的研究 | |||||
タイトル | ||||||
言語 | en | |||||
タイトル | Studies of Erythrocyte Membrane Lipid Abnormalities : Clinical and Experimental Studies to Elucidate the Relationship between Membrane Lipid Abnormality and Membrane Transport | |||||
言語 | ||||||
言語 | jpn | |||||
資源タイプ | ||||||
資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
資源タイプ | journal article | |||||
著者 |
橋本, 正志
× 橋本, 正志× Hashimoto, Masashi |
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著者所属(日) | ||||||
ja | ||||||
川崎医科大学血液内科 | ||||||
著者所属(英) | ||||||
en | ||||||
Division of Hematology, Department of Medicine, Kawasaki Medical School | ||||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | Membrane lipid abnormalities | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | Membrane transport | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | Phospholipase A2 | |||||
記事種別(日) | ||||||
内容記述タイプ | Other | |||||
内容記述 | 原著 | |||||
言語 | ja | |||||
抄録(日) | ||||||
ja | ||||||
赤血球膜の主たる構成々分である脂質あるいは蛋白の先天的・後天的異常による溶血性貧血症例において,赤血球膜輸送能の異常がしばしば認められる.また赤血球膜異常症に膜脂質異常を伴う場合がある.本研究では主に赤血球膜異常症諸種疾患の膜脂質分析と膜輸送能の相関を検討し,あわせて実験的に作製した膜脂質異常赤血球を検索することにより,赤血球膜異常症の病態を検討した.赤血球膜脂質異常症では,hereditary high red cell membrane phosphatidyl choline hemolytic anemia(HPCHA) 7例,congenital lecithin:cholesterol acyltransferase(LCAT) deficiency 1例,congenital β-lipoprotein(β-LP) deficiency 4例.膜蛋白異常症では,hereditary spherocytosis(HS),hereditary elliptocytosis(HE),hereditary stomatocytosis(HSt),およびその他疾患.実験的にはphospholipase A2(PLase A2)にて膜phosphatidyl choline(PC)に修飾を加えた膜脂質異常赤血球群を対象とした.先天性赤血球膜脂質異常症群では,HPCHA,LCAT欠損症で膜free cholesterol(FC)量・膜総phospholipids(PL)量共に著増,特にPC分画の著増が認められた.Na+ influxでは,HPCHAの著明な亢進に対しLCAT欠損症ではむしろ低値であった.β-LP欠損症では赤血球膜脂質総量はほぼ正常に維持されていたが,PC分画の減少とsphingomyelin分画の増加傾向が認められ,Na+ influxがほぼ正常であるのに対しCa2+ uptakeは他に類を見ないほど著明な亢進を示しており,本症での2者に著しい解離が注目された.赤血球膜蛋白異常症群においては,HSで摘脾前・後の変化が注目された.すなわち, 摘脾前の膜FC量・膜総PL量・phosphatidyl ethanolamine分画の減少が,摘脾後には正常化するが,Na+ influxの亢進は変化が認められず,病因の主体と考えられる膜蛋白の異常がNa+ influxの亢進に関与していると推定された.HEでは際立った異常は認められなかった.HStでは,膜脂質・膜輸送能とも各病型により異なる異常を呈するが両者間に相関は認められず,他の因子が関与しているものと推定された.PLase A2を用いる実験系では,無処理のcontrol群,PLase A2処理により膜PC減少とlyso-PC(L-PC)の増加を来した群,およびPLase A2処理後さらにalbumin処理を加え増加したL-PCを除去し膜総PL量も減少した群,の3群について膜PLとNa+ influxおよびCa2+ uptakeを測定した.この結果,膜脂質の変化とNa+ influxあるいはCa2+ uptakeの変化は明らかに相関を持つことが判明したが,PC・L-PC・総PL量のどの因子が主に関与しているかの特定には至らなかった.また,PLase A2処理赤血球のalbumin処理追加により,亢進したNa+ influxは変化を示さないのに比べ,Ca2+ uptakeでは更なる亢進を示し,NaとCaとではその輪送機構上に明らかな相違が存在することが判明した. | ||||||
抄録(英) | ||||||
en | ||||||
Membrane lipid analyses and membrane transport (Na+ influx and Ca2+ uptake) studies were performed in red cell of patients with membrane abnormalities. Among hereditary disorders with membrane lipid abnormalities, a marked increase of free cholesterol (FC) and of phosphatidyl choline (PC) was detected in the red cells of 7 cases of hereditary high red cell membrane PC hemolytic anemia (HPCHA) and one case of congenital lecithin : cholesterol acyltransferase (LCAT) deficiency. Na+ influx was elevated significantly in the HPCHA red cells, contrary to the decreased Na+ influx in LCAT deficiency. In 4 cases of congenital β-lipoprotein (β-LP) deficiency with a marked decrease of plasma lipids, total membrane lipids were maintained almost normally, even though a moderate decrement of PC and an increased sphingomyelin were observed. In the disorder, Ca2+ uptake was markedly increased (approximately 100 fold greater than those in normal control), with normal Na+ influx. Among red cell membrane protein abnormalities, moderate decrement of FC, total phospholipids (PL), and phosphatidyl ethanolamine in the non-splenectomized hereditary spherocytosis patients was normalized after splenectomy. Increased Na+ influx, however, was not improved after the treatment, suggesting a possible contribution of the abnormal membrane proteins to increased Na+ influx, if they may exist. In hereditary stomatocytosis, various membrane lipid abnormalities were observed dependent on the type of the disorders. To clarify a possible role of membrane lipids on the abnormal membrane transport functions, such as Na+ influx, normal red cells were treated with phospholipase A2 (PLase A2) to convert PC to lyso-PC (L-PC). In some experiments, the PLase A2-treated red cells were further incubated with albumin to cleave membrane L-PC, which was formed by the enzymatic degradation of membrane PC. In the PLase A2-treated red cells, Na+ influx and Ca2+ uptake were markedly elevated. Ca2+ uptake increases further in the presence of albumin, in contrast to no response in Na+ influx. In conclusion, (1) membrane lipid abnormalities do affect the membrane transport functions, and (2) a different mechanism would exist between Na+ influx and Ca2+ uptake. | ||||||
書誌情報 |
ja : 川崎医学会誌 en : Kawasaki medical journal 巻 13, 号 1, p. 30-43, 発行日 1987 |
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URL | ||||||
識別子 | http://igakkai.kms-igakkai.com/wp/wp-content/uploads/1987/KMJ13(1)30-43.1987.pdf | |||||
識別子タイプ | URI | |||||
DOI | ||||||
識別子タイプ | DOI | |||||
関連識別子 | https://doi.org/10.11482/KMJ-J13(1)30 | |||||
ISSN | ||||||
収録物識別子タイプ | PISSN | |||||
収録物識別子 | 0386-5924 | |||||
ISSN | ||||||
収録物識別子タイプ | EISSN | |||||
収録物識別子 | 2758-089X | |||||
雑誌書誌ID | ||||||
収録物識別子タイプ | NCID | |||||
収録物識別子 | AN00045593 | |||||
雑誌書誌ID | ||||||
収録物識別子タイプ | NCID | |||||
収録物識別子 | AN12940574 | |||||
著者版フラグ | ||||||
出版タイプ | VoR |