重度免疫不全マウス（C-NKG）

品系名：NOD/Shi-Prkdc^scid Il2rg^em1/Cyagen

バックグラウンド：NOD/Shi-Scid

品系作製策略：

C-NKGマウスはサイヤジェン株式会社がCRISPR/Cas9技術を使用してNOD/Shi-Scidバックグラウンド品系でIl2rg遺伝子をノックアウトした重症免疫不全マウスである。

品系の説明：

• 成熟したT、BとNK細胞を欠けている。
• 補体の活性が低い。
• マクロファージのヒト細胞への呑噬作用が弱い。
• 樹状細胞の機能が低い。
• T、B細胞の漏れがない。

研究応用：

• CDXとPDX。
• 細胞免疫療法の有効性と安全性の評価。
• 免疫系ヒト化マウスモデルの作製（ヒトPBMC或いはHSCを移植してヒト免疫系を再構築する）。
• GvHDモデル。
• 腫瘍免疫関連の研究。
• 造血と幹細胞に関する研究。

表現型の検証：

1. Prkdc遺伝子突然変異

Figure 1. Prkdc^scid mutation is produced by TAT → TAA in exon 84 of Prkdc gene. The sequencing result shows that NKG mice carry a Prkdc^scid mutation.

2. Il2rg遺伝子ノックアウト

Figure 2. The II2rg gene of NKG mouse was detected by PCR and the result shows that the II2rg gene of NKG mouse was successfully knocked out. The band size of wild type was 1430 bp and that of the knockout fragment was 388 bp.

3. C-NKGマウスの末梢血のB、T、NK細胞の検測

Figure 3. The peripheral blood of C-NKG mouse, a severe combined immunodeficiency(SCID) mouse model, is severely deficient in B, T and NK cells. Peripheral blood samples of BALB/c and C-NKG mice were collected, followed by the performance of flow cytometric immunophenotypic analysis and statistical comparison of the composition of T, B and NK cells. The results show that B cells (CD3^-CD19⁺), T cells (CD3⁺CD19^-), helper T cells (CD3⁺CD4⁺CD8^-), cytotoxic T cells (CD3⁺CD4^-CD8⁺) and NK cells (CD335⁺CD3^-) in the peripheral blood of C-NKG mice were almost completely absent while comparing with BALB/c mice.

4. C-NKGマウスのリンパ組織のB、T、NK細胞の検測

Figure 4. The lymphoid tissue of C-NKG mouse, a severe immunodeficiency mouse model, is severely deficient in B, T and NK cells. Lymphoid tissue samples of BALB/c and C-NKG mice were collected, followed by flow cytometric immunophenotypic analysis and statistical comparison of the composition of T, B and NK cells. The results show that B cells (CD3^-CD19⁺), T cells (CD3⁺CD19^-), helper T cells (CD3⁺CD4⁺CD8^-), cytotoxic T cells (CD3⁺CD4^-CD8⁺) and NK cells (CD335⁺CD3^-) in the lymphoid tissue of C-NKG mice were almost completely absent, as compared with BALB/c mice.