C1 esterase inhibitor

出典: meddic

補体C1
  • first aid step1 2006 p.187

USMLE

  • Q book p.193 33

臨床関連

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英文文献

  • Hereditary Angioedema Presenting as Recurrent Acute Pancreatitis.
  • Berger TD1, Garty BZ2.
  • Pediatrics.Pediatrics.2016 Jan 26. pii: peds.2015-0620. [Epub ahead of print]
  • Hereditary angioedema (HAE) may manifest with swelling of the face, extremities, and upper airways. Gastrointestinal symptoms are also common and may include abdominal pain, vomiting, and diarrhea. However, pancreatic involvement is rare and has been reported only in a few adults with previously dia
  • PMID 26812927
  • Acquired C1 esterase inhibitor deficiency in lymphomas: prevalence, symptoms, and response to treatment.
  • Bekos C1, Perkmann T2, Krauth M1, Raderer M3, Lechner K1, Jaeger U1.
  • Leukemia & lymphoma.Leuk Lymphoma.2016 Jan 21:1-4. [Epub ahead of print]
  • We retrospectively studied the prevalence of C1 esterase inhibitor (C1 INH) deficiency in 131 patients with various lymphomas. We determined C1 INH activity, C1 INH antigen, and C4 concentration at diagnosis and after chemotherapy. In follicular lymphoma (FL), diffuse large B-cell lymphoma (DLBCL) a
  • PMID 26795750
  • Nanofiltrated C1-esterase-inhibitor in the prophylactic treatment of bradykinin-mediated angioedema.
  • Greve J1, Hahn J1, Nordmann M1, Schuler PJ1, Bas M2, Hoffmann TK1, Hajdu Z2, Buchberger M2, Strassen U2.
  • Transfusion.Transfusion.2016 Jan 12. doi: 10.1111/trf.13462. [Epub ahead of print]
  • BACKGROUND: Patients suffering from bradykinin-induced angioedema show recurrent swelling of subcutaneous and submucosal structures. Increased bradykinin levels lead to an increase in vascular permeability and edema formation. Current therapy consists of B2 bradykinin receptor antagonists, C1-estera
  • PMID 26756974

和文文献

  • 水疱形成を伴った遺伝性血管性浮腫の1例
  • 遺伝性血管性浮腫(HAE) (特集 最近のトピックス2011) -- (最近話題の皮膚疾患)
  • 遺伝性血管性浮腫をめぐる諸問題

関連リンク

C1 esterase inhibitor (C1-INH) is a protein found in the fluid part of your blood that controls C1, the first component of the complement system. The complement system is a group of proteins that move freely through your ...
Definition C1 esterase inhibitor (C1-INH) is a protein found in the fluid part of your blood that controls C1, the first component of the complement system. The complement system is a group of proteins that move freely through your ...

関連画像

Hereditary Angioedema and AnesthesiaAcquired C1 esterase inhibitor deficiency C1 Esterase Inhibitor Deficiency submited  due to C1 esterase inhibitor deficiency ® (C1 esterase inhibitor [human]) worksAcquired C1 Esterase Inhibitor Deficiency


★リンクテーブル★
先読み補体
リンク元C1エステラーゼインヒビター
関連記事inhibit」「C」「inhibitor」「C1」「esterase

補体」

  [★]

complement
アレキシン alexin
補体活性化経路補体価


  • 図:IMM.63
pathway start first step
classical pathway antigen-antibody complex C1q, C1r, C1s, C4, C2
lectin pathway mannose-binding lectin or ficolin binds carbohydrate on pathogen surface MBL/ficolin, MASP-2, C4, C2
alternative pathway pathogen surface C3, B, D

補体の相互作用 IMM.62

  • C1qがカスケードの最初となる。3つの方法で補体カスケードがはじまる。
  • (1)多価陰イオン表面(例えば、グラム陰性細菌のリポテイコ酸)
  • (2)バクテリアの多糖のホスホコリンに結合(例えば肺炎球菌のC蛋白質)
  • (3)抗原抗体複合体に結合して自然免疫と獲得免疫のエフェクター機構を結びつける。
  • 炭化水素鎖を認識するレクチンがカスケードの最初となる。
  • C3からはじまる。血漿中のC3が病原体の表面で自発的に活性化されることで補体反応がはじまる。
3つの経路は共通してC3 convertaseを生成。C3 convertaseはC3→C3a+C3b
C3a: C3a is a peptide mediator of local inflammation
C3b: C3b binds covalently to the bacterial cell membrane and opsonizes the bacteria
C5 convertaseはC3bにC3 convertaseが結合してできる
C5 converaseはC5→C5a+C5b
C5a: powerful peptide mediator of inflammation
C5b: C5b,C6,C7,C8,C9: membrane-attack complex

C3aとC5a。C4a

炎症部位に血管外に抗体、補体、食細胞を集め、組織液を増やすことで抗原提示細胞がリンパ節に移動するのを促進する(IMM.75)
  • C5a,C3a,C4a: smooth muscle contraction.(IMM.75)
  • C5a,C3a: act on the endo thelial cells lining blood vessels to induce adhesion molecules.(IMM.75)
  • C5a>C3a>C4a: increase in blood flow, increase vascular permeability, increase binding of phagocytes to exdothielial cells.(IMM.76)
  • C5a: activates mast cells to release mediators, such as histamine and TNF-α. that contribute the inflammatory response(IMM.76).
  • C5a: acts directly on neutrophils and monocytes and attracting neutrophils and monocytes(IMM.75)
  • C3a: contributes to the pypotension and edema seen in endotoxic shock
  • C5a: activated by endotoxin, funcions in neutrophil chemotaxis

C5a

臨床関連

  • 増加:



C1エステラーゼインヒビター」

  [★]

C1 esterase inhibitor
C1

C1インヒビター ← 現在ではこちらがよく使われる。

  • first aid step1 2006 p.187


inhibit」

  [★]

  • v.
  • 抑制する、阻害する、阻止する
abrogateblockdepressdepressiondeterinhibitioninterdictpreventpreventionrepressrepressionrestrainrestraintsuppresssuppression

WordNet   license wordnet

「limit, block, or decrease the action or function of; "inhibit the action of the enzyme"; "inhibit the rate of a chemical reaction"」

PrepTutorEJDIC   license prepejdic

「〈感情・欲望・行動・作用など〉‘を'抑制する / (…しないように)〈人〉‘を'抑制する,妨げる《+『名』+『from』+『名』(do『ing』)》」

WordNet   license wordnet

「control and refrain from showing; of emotions, desires, impulses, or behavior」
bottle up, suppress

WordNet   license wordnet

「limit the range or extent of; "Contact between the young was inhibited by strict social customs"」


C」

  [★]

WordNet   license wordnet

「the 3rd letter of the Roman alphabet」
c

WordNet   license wordnet

「(music) the keynote of the scale of C major」

WordNet   license wordnet

「a general-purpose programing language closely associated with the UNIX operating system」

PrepTutorEJDIC   license prepejdic

「carbonの化学記号」


inhibitor」

  [★]

  • n.
blockerdepressantsuppressant

WordNet   license wordnet

「a substance that retards or stops an activity」

PrepTutorEJDIC   license prepejdic

「抑制する人(物) / 化学反応抑制剤」


C1」

  [★]


esterase」

  [★] エステラーゼエステル加水分解酵素エステル分解酵素




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