hC3

品系全名

C57BL/6JSmo-C3tm1(hC3)/Smoc

目录号

NM-HU-2000079

品系状态

活体

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基因信息

基因名
C3

基因曾用名

ASP; Plp; HSE-MSF; AI255234

NCBI ID

12266

MGI ID

88227

Ensembl ID

ENSMUSG00000024164

Pubmed

C3

人类同源基因

C3

品系描述

通过同源重组，将小鼠C3基因进行人源化修饰。 风险提示：由于人源化C3 蛋白与小鼠补体调节因子的相互作用失调，hC3 小鼠会出现类似 C3G 患者的病理特征，纯合子小鼠死亡率高（约在10周龄时出现死亡，至15周龄左右，死亡率达到一半），不同批次小鼠死亡率和死亡时间可能存在差异。
应用领域：免疫治疗；药物筛选

验证数据

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Fig1. Detection of C3 expression in liver by RT-PCR. 

Wild type: only one band at 359 bp with primers F1/R1(mC3);

Heterozygous: one band at 359 bp with primers F1/R1(mC3) and one band at 357 bp with primers F2/R2(hC3); 

Abbr. M, DNA marker; HO, homozygous; HE, heterozygous; WT, wild type.

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Fig 2. Detection of heterozygous hC3 expression in serum by ELISA. 

Abbr. Het, heterozygous; WT, wild type.

Note: Data from the collaborator.

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Fig 3. Detection of hC3 expression in serum by ELISA(n=3).


Note: The human Complement C3 ELISA Kit (ab108823) specifically recognized humanized C3.

Abbr. HO, homozygous; WT, wild type.

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Fig 4. Detection of hC3 expression in eyes in HO hC3 KI mice by IF (n=5/group).

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Fig 5. The HO hC3 mice showed inflammation in the portal region and bile duct hyperplasia (100× and 400×)

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Fig 6. The HO hC3 mice showed glomerular sclerosis, glomerular atrophy and inflammation of the capillary tufts area (100× and 400×)

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Fig 7. Body Weight of HO hC3 mouse (n=12/group).

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Fig 8. Survival curve of HO hC3 mouse (n=12/group).

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Fig 9. Serum Biochemistry of HO hC3 mouse (n=5/group).

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Fig 10. Serum Biochemistry of HO hC3 mouse (n=5/group, a different biochemical analyzer was used than in Fig 9).

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Fig 11. Urinalysis of HO hC3 mouse.

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Fig 12. Single dose of ARO-C3 reduced serum hC3 levels of HO hC3 mouse (n=7-8/group).

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Fig 13. Survival curve and body weight of HO hC3 mouse post ARO-C3 treatment (n=7-8/group).

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