Background:

• Hereditary angioedema (HAE) has three main types: type I, type II, and type III. Among them, types I and II are caused by mutations in the SERPING1 gene, resulting in deficiency (type I) or dysfunction (type II) of complement C1 inhibitor (C1-INH). Types I and II account for approximately 99% of all cases.
• The figure depicts the core regulatory role of C1 esterase inhibitor (C1-INH) in the kallikrein-kinin system (KKS): C1-INH controls KKS activation and bradykinin (BK) production by inhibiting FXII activation, plasma kallikrein (PK) activity, and the reciprocal activation between FXII and PK (including the plasmin-mediated amplification loop).

Targeting strategy:

• The exons 1~8 of mouse Serping1 gene were knocked out in B-Serping1 KO mice, leading to a complete loss of Serping1 protein expression in B-Serping1 KO mice.

Verification: 

• Mouse Serping1 mRNA was only detectable in wild-type mice, but not in homozygous B-Serping1 KO mice.

Application:

• This product can be used to study the drug screening and mechanism research of hereditary angioedema (HAE) and other related diseases, and study the complement system, coagulation abnormalities and the development of anti-inflammatory drugs.

Gene targeting strategy for B-Serping1 KO mice. The exons 1~8 of mouse Serping1 gene were knocked out in B-Serping1 KO mice.

Strain specific analysis of mSerping1 mRNA expression in wild-type C57BL/6JNifdc mice and B-Serping1 KO mice by RT-PCR. Liver RNA were isolated from wild-type C57BL/6JNifdc mice (+/+) and homozygous B-Serping1 KO mice (-/-), then cDNA libraries were synthesized by reverse transcription, followed by PCR with mouse Serping1 primers. Mouse Serping1 mRNA was only detectable in wild-type mice but not in homozygous B-Serping1 KO mice.