C1 Esterase inhibitor from human plasma
SIGMA/SRP6317 - ≥95% (SDS-PAGE)
Synonym: C1-inhibiting factor; Complement C1 esterase inhibitor; Esterase inhibitor C-1; Plasma protease C1 inhibitor; Serpin G1
Product Type: Chemical
Immunoblotting C1 Esterase inhibitor, human Plasma Cat. No. SRP6317, 4-12% Bis-Tris NuPAGE gel of C1 Esterase inhibitor, human Plasma. Lanes 1. C1 Esterase Inhibitor - 2.5 μg (reduced/heated) 2. C1 Esterase Inhibitor - 5 μg (reduced/heated) 3. C 1 Esterase Inhibitor - 10 μg (reduced/heated) 4. BRMW Standard 5. C1 Esterase Inhibitor - 2.5 μg (non-reduced/heated) 6. C1 Esterase Inhibitor - 5 μg (non-reduced/heated) 7. C1 Esterase Inhibitor - 10 μg (non-reduced/heated) 8. C1 Esterase Inhibitor - 5 μg (non-reduced/no heat)
| assay | ≥95% (SDS-PAGE) |
| biological source | human |
| form | frozen liquid |
| mol wt | 100 kDa |
| packaging | pkg of 1 mg |
| shipped in | dry ice |
| storage temp. | −70°C |
| UniProt accession no. | P05155   |
| Biochem/physiol Actions: | C1 esterase inhibitor functions as a serine proteinase inhibitor. The concentration of C1 esterase inhibitor protein is reduced to 10-30% of normal in patients with angioedema secondary to C1 esterase inhibitor deficiency (85% of patients with Hereditary Angioedema (HAE)); in 15% of patients with HAE, the concentrations of the inhibitor protein is normal but function is markedly reduced. C1 esterase inhibitor deficiency is a rare condition resulting in facial swelling and abdominal cramping. Usually the condition is hereditary, though it may also occur when the protein is non-functional. C1 esterase inhibitor deficiencies also disturb the fibrinolytic system, the intrinsic coagulation pathway and the complement pathway. |
| General description: | C1 esterase inhibitor is a single chain glycoprotein which inhibits C1, C1r, C1s, plasma kallikrein, factors XIa, XIIa and plasmin of the blood clotting system. It is present in the plasma at 16-33 mg/100mL. It is part of the serpin family. |
| Physical form: | Frozen in 20 mM potassium phosphate, pH 7.0, with 250 mM KCl. |
| RIDADR | NONH for all modes of transport |
| WGK Germany | WGK 3 |
| Flash Point(F) | Not applicable |
| Flash Point(C) | Not applicable |
| Purity | ≥95% (SDS-PAGE) |
| Storage Temp. | −70°C |
| UNSPSC | 12352200 |