BP180

出典: meddic

XVII型コラーゲン、180kDa類天疱瘡抗原


水疱性類天疱瘡の治療効果判定

  • 抗BP180抗体の血清レベルは水疱性類天疱瘡の病勢を反映している。

臨床関連


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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2013/12/27 21:28:39」(JST)

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英文文献

  • Immunopathological characteristics of patients with bullous pemphigoid and neurological disease.
  • Taghipour K1, Chi CC, Bhogal B, Groves RW, Venning V, Wojnarowska F.Author information 1Division of Genetics and Molecular Medicine, King's College, London, UK; Department of Dermatology, Oxford University Hospitals, Oxford, UK.AbstractBACKGROUND: The relationship between bullous pemphigoid (BP) and neurological disease has been the subject of numerous recent studies and BP antigens and their isoforms have been identified in the central nervous system (CNS). Whilst epidemiological data support this association, little is known about the pathomechanism behind this link and the immunological characteristics of patients with BP and neurological disease, other than multiple sclerosis (MS), has not been studied.
  • Journal of the European Academy of Dermatology and Venereology : JEADV.J Eur Acad Dermatol Venereol.2014 May;28(5):569-73. doi: 10.1111/jdv.12136. Epub 2013 Mar 26.
  • BACKGROUND: The relationship between bullous pemphigoid (BP) and neurological disease has been the subject of numerous recent studies and BP antigens and their isoforms have been identified in the central nervous system (CNS). Whilst epidemiological data support this association, little is known abo
  • PMID 23530989
  • Isolation of a hemidesmosome-rich fraction from a human squamous cell carcinoma cell line.
  • Hirako Y1, Yonemoto Y2, Yamauchi T2, Nishizawa Y3, Kawamoto Y3, Owaribe K2.Author information 1Division of Biological Science, Graduate School of Science, Nagoya University, Furo-cho, Chikusa-ku, Nagoya 464-8602, Japan. Electronic address: s47526a@cc.nagoya-u.ac.jp.2Division of Biological Science, Graduate School of Science, Nagoya University, Furo-cho, Chikusa-ku, Nagoya 464-8602, Japan.3Department of Biomedical Sciences, Chubu University, 1200 Matsumoto-cho, Kasugai 487-8501, Japan.AbstractHemidesmosomes are cell-to-matrix adhesion complexes anchoring keratinocytes to basement membranes. For the first time, we present a method to prepare a fraction from human cultured cells that are highly enriched in hemidesmosomal proteins. Using DJM-1 cells derived from human squamous cell carcinoma, accumulation of hemidesmosomes was observed when these cells were cultured for more than 10 days in a commercial serum-free medium without supplemental calcium. Electron microscopy demonstrated that numerous electron-dense adhesion structures were present along the basal cell membranes of DJM-1 cells cultured under the aforementioned conditions. After removing cellular materials using an ammonia solution, hemidesmosomal proteins and deposited extracellular matrix were collected and separated by electrophoresis. There were eight major polypeptides, which were determined to be plectin, BP230, BP180, integrin α6 and β4 subunits, and laminin-332 by immunoblotting and mass spectrometry. Therefore, we designated this preparation as a hemidesmosome-rich fraction. This fraction contained laminin-332 exclusively in its unprocessed form, which may account for the promotion of laminin deposition, and minimal amounts of Lutheran blood group protein, a nonhemidesmosomal transmembrane protein. This hemidesmosome-rich fraction would be useful not only for biological research on hemidesmosomes but also for developing a serum test for patients with blistering skin diseases.
  • Experimental cell research.Exp Cell Res.2014 Apr 12. pii: S0014-4827(14)00148-7. doi: 10.1016/j.yexcr.2014.04.002. [Epub ahead of print]
  • Hemidesmosomes are cell-to-matrix adhesion complexes anchoring keratinocytes to basement membranes. For the first time, we present a method to prepare a fraction from human cultured cells that are highly enriched in hemidesmosomal proteins. Using DJM-1 cells derived from human squamous cell carcinom
  • PMID 24726610
  • Dual roles of hemidesmosomal proteins in the pancreatic epithelium: the phosphoinositide 3-kinase decides.
  • Laval S1, Laklai H1, Fanjul M1, Pucelle M2, Laurell H1, Billon-Galés A1, Le Guellec S3, Delisle MB3, Sonnenberg A4, Susini C1, Pyronnet S1, Bousquet C1.Author information 11] INSERM UMR 1037, Laboratoire d'excellence Toulouse Cancer (labex TOUCAN), Equipe labellisée Ligue Nationale Contre le Cancer (LNCC), Centre de Recherche en Cancérologie de Toulouse (CRCT), Toulouse, France [2] Université Toulouse III Paul Sabatier, Toulouse, France.2INSERM UMR 1037, Laboratoire d'excellence Toulouse Cancer (labex TOUCAN), Equipe labellisée Ligue Nationale Contre le Cancer (LNCC), Centre de Recherche en Cancérologie de Toulouse (CRCT), Toulouse, France.3Services d'Anatomie et Cytologie Pathologique of Hôpital Rangueil, Toulouse, France.4Department of Cell Biology, The Netherlands Cancer Institute, Amsterdam, The Netherlands.AbstractGiven the failure of chemo- and biotherapies to fight advanced pancreatic cancer, one major challenge is to identify critical events that initiate invasion. One priming step in epithelia carcinogenesis is the disruption of epithelial cell anchorage to the basement membrane which can be provided by hemidesmosomes (HDs). However, the existence of HDs in pancreatic ductal epithelium and their role in carcinogenesis remain unexplored. HDs have been explored in normal and cancer pancreatic cells, and patient samples. Unique cancer cell models where HD assembly can be pharmacologically manipulated by somatostatin/sst2 signaling have been then used to investigate the role and molecular mechanisms of dynamic HD during pancreatic carcinogenesis. We surprisingly report the presence of mature type-1 HDs comprising the integrin α6β4 and bullous pemphigoid antigen BP180 in the human pancreatic ductal epithelium. Importantly, HDs are shown to disassemble during pancreatic carcinogenesis. HD breakdown requires phosphoinositide 3-kinase (PI3K)-dependent induction of the matrix-metalloprotease MMP-9, which cleaves BP180. Consequently, integrin α6β4 delocalizes to the cell-leading edges where it paradoxically promotes cell migration and invasion through S100A4 activation. As S100A4 in turn stimulates MMP-9 expression, a vicious cycle maintains BP180 cleavage. Inactivation of this PI3K-MMP-9-S100A4 signaling loop conversely blocks BP180 cleavage, induces HD reassembly and inhibits cell invasion. We conclude that mature type-1 HDs are critical anchoring structures for the pancreatic ductal epithelium whose disruption, upon PI3K activation during carcinogenesis, provokes pancreatic cancer cell migration and invasion.
  • Oncogene.Oncogene.2014 Apr 10;33(15):1934-44. doi: 10.1038/onc.2013.146. Epub 2013 Apr 29.
  • Given the failure of chemo- and biotherapies to fight advanced pancreatic cancer, one major challenge is to identify critical events that initiate invasion. One priming step in epithelia carcinogenesis is the disruption of epithelial cell anchorage to the basement membrane which can be provided by h
  • PMID 23624916

和文文献

  • 自己免疫性水疱症の診断戦略 (AYUMI 自己免疫性水疱症--今日の進歩)
  • 症例報告 水疱形成に乏しい妊娠性疱疹の1例
  • 花輪 書絵,安藤 典子,原田 和俊 [他]
  • 臨床皮膚科 65(1), 8-11, 2011-01
  • NAID 40018270339

関連リンク

BP180の主要なエピトープは、NC16aと呼ばれる最も細胞膜に近い部分に存在し、大部分の患者血清がこの部位の組み替え蛋白質に反応性を示す。本試薬は抗原としてBP180NC16aを用いて、患者血清中の抗BP180抗体を特異的に測定する ...
抗BP180抗体 - 水疱性類天疱瘡の診断および病勢モニタリングに有用な検査です。 ... この疾患は、表皮基底膜細胞と基底膜の結合に関するヘミデスモゾームの構成タンパクに対する自己抗体によって発症するもので、標的抗原は主に230kD ...

関連画像

 Air Pumps > Million Air Battery Pump BP180Figure 1 . Schematic representation of the  -BEARCAT-SPORTCAT-BP120-BP150-BP180-BP250MESACUP™ BP180 テスト (7695) | 製品 BP180 Battery for Uniden SC 150 BC 230 BC Pics collge girls nipples black girls with


★リンクテーブル★
先読み基底板
リンク元類天疱瘡」「天疱瘡」「妊娠性疱疹」「水疱性類天疱瘡」「基底膜

基底板」

  [★]

basal lamina, (違う気がする。要調査→)lamina lucida (NDE)
lamina basalis
基底膜

表皮の基底細胞の深層



類天疱瘡」

  [★]

pemphigoid
水疱症


  • 自己抗体
  • 表皮下水疱 → 緊満性水疱

表皮下水疱症(類天疱瘡群) (NDE.218)

  • 自己免疫性水疱症(後天性水疱症) autoimmuno blistering disease
  • 表皮下水疱症(類天疱瘡群)


代表的な天疱瘡と類天疱瘡の比較

  尋常性天疱瘡 落葉性天疱瘡 水疱性類天疱瘡
年齢 中年~老年 中年 老年(若年もあり)
好発部位 口腔粘膜全身 全身 全身
臨床像 皮膚所見 水疱びらん びらん、葉状落屑、 緊満性水疱浮腫性紅斑掻痒
粘膜浸潤 ++ 痂皮
Nikolsky現象  
病理組織像 所見 表皮内水疱
(棘融解)
表皮下水疱
好酸球浸潤
Tzanck試験  
棘融解部位 表皮下層(基底細胞直上) 表皮上層(顆粒層)  
抗原 Dsg3 のみ、
Dsg3 と1 の共存
Dsg1 のみ BP180BP230
ELISA Dsg1(+または-)、Dsg3(+) Dsg1(+)、Dsg3(-)  
蛍光抗体法所見 直接法(病変部皮膚) 表皮細胞間に
IgGC3 陽性
病変部基底膜部に
IgGC3 の線状沈着
間接法(血清中) 抗表皮細胞間物質抗体(IgG)陽性 抗基底膜抗体検出
治療 ステロイド免疫抑制薬血漿交換療法、γ グロブリン療法 ステロイド内服免疫抑制薬DDSなど



天疱瘡」

  [★]

pemphigus
水疱症

概念

  • 表皮細胞間物質に対する自己抗体によって棘融解が生じる自己免疫疾患(NDE.211)であり、皮膚・粘膜における著明な水疱形成を特徴とする。

病因

  • 表皮細胞間物質に対する自己抗体による。

疫学

  • 多くは中高年に好発するが、新生児に特異的、腫瘍に随伴、薬物に誘発、あるいは地域特性がある天疱瘡がある。

病理

代表的な天疱瘡と類天疱瘡の比較

  尋常性天疱瘡 落葉性天疱瘡 水疱性類天疱瘡
年齢 中年~老年 中年 老年(若年もあり)
好発部位 口腔粘膜、全身 全身 全身
臨床像 皮膚所見 水疱、びらん びらん、葉状落屑、 緊満性水疱、浮腫性紅斑、掻痒
粘膜浸潤 ++ 痂皮
Nikolsky 現象  
病理組織像 所見 表皮内水疱
(棘融解)
表皮下水疱
好酸球の浸潤
Tzanck 試験  
棘融解部位 表皮下層(基底細胞直上) 表皮上層(顆粒層)  
抗原 Dsg3 のみ、
Dsg3 と1 の共存
Dsg1 のみ BP180BP230
ELISA Dsg1(+または-)、Dsg3(+) Dsg1(+)、Dsg3(-)  
蛍光抗体法所見 直接法(病変部皮膚) 表皮細胞間に
IgG、C3 陽性
病変部基底膜部に
IgG とC3 の線状沈着
間接法(血清中) 抗表皮細胞間物質抗体(IgG)陽性 抗基底膜抗体の検出
治療 ステロイド、免疫抑制薬、血漿交換療法、γ グロブリン療法 ステロイド内服、免疫抑制薬、DDSなど

参考

  • 1. [charged] Pemphigus - uptodate [1]




妊娠性疱疹」

  [★]

herpes gestationis HG, hydroa gestationis, herpes circinatus bullosa
妊娠ヘルペス妊娠性類天疱瘡 pemphigoid gestationis
表皮下水疱症

概念

  • 妊娠中・産褥期(妊娠4か月-分娩直後)に生じる水疱性類天疱瘡類似疾患

疫学

  • 分娩5000-10000に1回

病因

病理

症状

  • 膨疹様紅斑のまわりに小水疱が形成される
  • 発生部位:腹部、殿部、四肢

治療

  • 軽症例:副腎皮質ステロイド外用
  • 重症例:副腎皮質ステロイド内服

予後

  • 出産後2-3 か月で消退
  • 妊娠ごとに反復、重症化しやすい



水疱性類天疱瘡」

  [★]

bullous pemphigoid BP
類天疱瘡


概念

病因

病理

  • 表皮下水疱

蛍光抗体法

  • 基底膜に一致した免疫グロブリンの沈着

参考

  • 1. [charged] Bullous pemphigoid and other pemphigoid disorders - uptodate [2]


基底膜」

  [★]

basilar membrane (Z), basement membrane, BM
membrana basalis
基底板× basal lamina×




  • 図:NDE.6(シェーマ)


  • 表皮直下に存在。光学顕微鏡下でPAS染色に赤色に染まり、ジアスターゼ抵抗性を示す。電顕的には複雑な構造を示す。(NDE.6)

基底膜近傍の構造

  • BP180



"http://meddic.jp/BP180" より作成


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