von Willebrand factor

https://www.uniprot.org/uniprot/P04275

  • Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex GPIb-IX-V. Also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clearance from plasma.

血漿中にある

thrombomodulin

https://www.uniprot.org/uniprot/P07204

  • Thrombomodulin is a specific endothelial cell receptor that forms a 1:1 stoichiometric complex with thrombin. This complex is responsible for the conversion of protein C to the activated protein C (protein Ca). Once evolved, protein Ca scissions the activated cofactors of the coagulation mechanism, factor Va and factor VIIIa, and thereby reduces the amount of thrombin generated.

内皮細胞上のトロンビン受容体。プロテインCを活性型に変換する。プロテインCaはVa、VIIIaを消化し、トロンビンの生成を抑制的に調節する。

第XII因子

https://www.uniprot.org/uniprot/P00748

  • Factor XII is a serum glycoprotein that participates in the initiation of blood coagulation, fibrinolysis, and the generation of bradykinin and angiotensin. Prekallikrein is cleaved by factor XII to form kallikrein, which then cleaves factor XII first to alpha-factor XIIa and then trypsin cleaves it to beta-factor XIIa. Alpha-factor XIIa activates factor XI to factor XIa.

血清の糖タンパクで血液凝固の開始、線溶系、ブラジキニンとアンギオテンシンの生成に関わる。

  • Selective cleavage of Arg-|-Ile bonds in factor VII to form factor VIIa and factor XI to form factor XIa. EC:3.4.21.38

アルギニンとイソロイシンの間を切断し、第VII因子と第XI因子を活性化する。

  • Factor XII is activated by kallikrein in alpha-factor XIIa, which is further converted by trypsin into beta-factor XIIa. Alpha-factor XIIa is composed of an NH2-terminal heavy chain, called coagulation factor XIIa heavy chain, and a COOH-terminal light chain, called coagulation factor XIIa light chain, connected by a disulfide bond. Beta-factor XIIa is composed of 2 chains linked by a disulfide bond, an N-terminal nonapeptide, called beta-factor XIIa part 1, and coagulation factor XIIa light chain, also known in this context as beta-factor XIIa part 2.

カリクレインにより活性化され(α第XIIa因子)さらにトリプシンによりβ第XIIa因子に変換される。αはN末端側の重鎖とC末端側の軽鎖がジスルフィド結合でつながる。

  • O- and N-glycosylated. The O-linked polysaccharides were not identified, but are probably the mucin type linked to GalNAc.

ステロール調節エレメント結合タンパク質

コレステロールやその代謝産物は、転写因子「ステロール調節エレメント結合タンパク質」(SREBP)を介して、HMG-CoAレダクターゼ遺伝子の転写を抑制する。
LDL受容体遺伝子のプロモーター領域に存在し、ステロール欠乏下に遺伝子転写を促進し、ステロールによって転写活性を低下させる。
AMPKによるリン酸化を受けて、プロセッシングと核移行が抑制される。結果として標的遺伝子の発現を抑制する。
SREBP-1は脂肪酸代謝、SREBP−2はコレステロール代謝に関与する。
小胞体コレステロール諒が低下すると、SREBP-2はプロセッシングを受けて核へ移行し、転写因子として応答遺伝子発現を調節する。

VLCAD; Very long-chain specific acyl-CoA dehydrogenase

パルミトイル(ミリストイル、ステアロイル)-CoAなど長鎖脂肪酸エステルに対し活性をもつ。炭素数24までのアシル基を基質としうるが、炭素数12以下はほとんど活性を示さない。
ミトコンドリア脂肪酸β酸化経路に含まれる。
他に、

ACADSB; Short/branched chain specific acyl-CoA dehydrogenase

短鎖(ブチリルCoA、ヘキサノイルCoA)または分枝鎖(2-メチルブチリルCoA、2-メチルヘキサノイルCoA)の代謝に高い活性をもつ。
バルプロ酸代謝にも関与する。

ACADS; Short-chain specific acyl-CoA dehydrogenase

素数6以下のアシルCoAのβ酸化に関与する。

ACADM: Medium-chain specific acyl-CoA dehydrogenase

素数4-16のアシルCoAのβ酸化に関与する。

ACADL; Long-chain specific acyl-CoA dehydrogenase

ヒアルロン酸の分解

Hyaluronidase-2

www.uniprot.org

Function

Hydrolyzes high molecular weight hyaluronic acid to produce an intermediate-sized product which is further hydrolyzed by sperm hyaluronidase to give small oligosaccharides. Displays very low levels of activity. Associates with and negatively regulates MST1R.

Cell migration-inducing and hyaluronan-binding protein

www.uniprot.org
KIAA1199

Function

クラスリンによるエンドサイトーシスでヒアルロナンを分解する。ヒアルロン酸に結合する。中程度のサイズに分解する。
Mediates depolymerization of hyaluronic acid (HA) via the cell membrane-associated clathrin-coated pit endocytic pathway. Binds to hyaluronic acid. Hydrolyzes high molecular weight hyaluronic acid to produce an intermediate-sized product, a process that may occur through rapid vesicle endocytosis and recycling without intracytoplasmic accumulation or digestion in lysosomes. Involved in hyaluronan catabolism in the dermis of the skin and arthritic synovium. Positively regulates epithelial-mesenchymal transition (EMT), and hence tumor cell growth, invasion and cancer dissemination. In collaboration with HSPA5/BIP, promotes cancer cell migration in a calcium and PKC-dependent manner. May be involved in hearing.

Activity

Random hydrolysis of (1->4)-linkages between N-acetyl-beta-D-glucosamine and D-glucuronate residues in hyaluronate.

Cell surface hyaluronidase

www.uniprot.org

CEMIP2, TMEM2

Function

Cell surface hyaluronidase that mediates the initial cleavage of extracellular high-molecular-weight hyaluronan into intermediate-size hyaluronan of approximately 5 kDa fragments (PubMed:28246172). Acts as a regulator of angiogenesis and heart morphogenesis by mediating degradation of extracellular hyaluronan, thereby regulating VEGF signaling (By similarity). Is very specific to hyaluronan; not able to cleave chondroitin sulfate or dermatan sulfate (PubMed:28246172).

activity

Random hydrolysis of (1->4)-linkages between N-acetyl-beta-D-glucosamine and D-glucuronate residues in hyaluronate.

Cofactor

Ca2+