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特發性肺纖維化（IPF）是一種慢性、進行性、致死性的間質性肺炎，以肺組織不可逆性纖維化重塑為特征，最終導致呼吸衰竭。其核心病理機制包括肺泡上皮細胞反復損傷、異常修復，驅動成纖維細胞活化為肌成纖維細胞，導致細胞外基質過度沉積，形成肺瘢痕。臨床表現為活動性呼吸困難、干咳、疲勞，常伴肺功能進行性下降，約50%患者合并間質性肺病（ILD）。IPF主要影響50歲以上人群，中位生存期僅2-5年，預后差于多數癌癥，故被稱為"不是癌癥的癌癥"。?

特發性肺纖維化（IPF）的藥物研發聚焦于纖維化、炎癥及細胞修復等核心機制，主要關注以下靶點：

● 轉化生長因子-β（TGF-β）通路：現有藥物尼達尼布通過抑制TGF-β下游受體（VEGFR、PDGFR、FGFR）延緩肺功能下降。百時美施貴寶的BMS-986278（Admilparant）作為溶血磷脂酸受體1（LPAR1）拮抗劑，其II期研究顯示可降低FVC年下降率54%，目前III期試驗正在進行中。

● 磷酸二酯酶 4B（PDE4B）：勃林格殷格翰的口服抑制劑nerandomilast在III期臨床試驗 FIBRONEER-IPF中顯著減緩FVC下降，且安全性可控，已在中國提交上市申請并被納入優先審評。

● 結締組織生長因子（CTGF）：Pliant Therapeutics的Bexotegrast（PLN-74809）通過抑制CTGF與TGF-β結合，在IIa期試驗中顯示FVC下降幅度減少，目前進入IIb/III期臨床。

目前，特發性肺纖維化新藥研發正朝著多靶點與新型機制方向突破。英矽智能借助AI發現TNIK激酶抑制劑INS018_055，臨床前展現優于尼達尼布的抗纖維化效果，且與吡非尼酮聯用增效；北京生命科學研究所鎖定AREG蛋白為關鍵靶點，其抑制劑已進入一期臨床。細胞療法領域，上海交通大學的自體基底干細胞移植、吉美瑞生的REGEND001氣道基底層干細胞療法均在臨床試驗中取得肺功能改善等積極成果。此外，韓國研究證實依折麥布可抑制成纖維細胞分化，中國學者發現GOLM1-KLF4-NEAT1信號通路具備潛在價值，為IPF治療帶來新希望。

特發性肺纖維化在研藥物靶點

特發性肺纖維化藥物靶點相關產品推薦

● 靶點蛋白

CCN2
CSB-MP5112MOW

Measured by its binding ability in a functional ELISA. Immobilized Rhesus macaque CTGF at 1 μg/ml can bind Anti-CTGF recombinant antibody (CSB-RA006147MA2HU). The EC₅₀ is 0.6886-0.7825 ng/mL.

CD274
CSB-MP878942HU1

Measured by its binding ability in a functional ELISA. Immobilized PD-L1 at 2 μg/ml can bind Anti- PD-L1 mouse monoclonal antibody(CSB-MA878942A1m,antigen from E.coli), the EC₅₀ of human PD-L1 protein is 1.252-1.653 ng/mL.

CXCR4
CSB-MP006254HU(F1)

Measured by its binding ability in a functional ELISA. Immobilized Human CXCR4 at 10 μg/ml can bind Anti-CXCR4 recombinant antibody (CSB-RA006254MA01HU), the EC₅₀ is 101.7-253.6 ng/mL.

NT5E
CSB-MP723415HU

Measured by its binding ability in a functional ELISA. Immobilized CD73 at 2 μg/ml can bind Anti- CD73 Rabbit Monoclonal Antibody(CSB-RA978310A0HU), the EC₅₀ is 3.212-4.525 ng/ml.

Target	Product Name	Source	Tag Info	Code
CCL2	Recombinant Human C-C motif chemokine 2 (CCL2)	E.coli	N-terminal GST-tagged	CSB-EP004783HU
CCL2	Recombinant Human C-C motif chemokine 2 (CCL2)	E.coli	N-terminal 10xHis-tagged and C-terminal Myc-tagged	CSB-EP004783HUb1
CCL2	Recombinant Mouse C-C motif chemokine 2 (Ccl2), partial	E.coli	N-terminal 6xHis-tagged	CSB-EP004783MO
CCN2	Recombinant Human CCN family member 2 (CCN2)	E.coli	C-terminal 6xHis-tagged	CSB-EP006147HU
CCN2	Recombinant Human CCN family member 2 (CCN2), partial	E.coli	N-terminal 6xHis-tagged	CSB-EP006147HU1
Ccn2	Recombinant Mouse CCN family member 2 (Ccn2), partial	E.coli	N-terminal 10xHis-SUMO-tagged	CSB-EP006147MO
Ccn2	Recombinant Mouse CCN family member 2 (Ccn2), partial	E.coli	N-terminal GST-tagged	CSB-EP006147MOe0
CCN2	Recombinant Macaca mulatta CCN family member 2 (CCN2) (Active)	Mammalian cell	C-terminal 10xHis-tagged	CSB-MP5112MOW
Ccn4	Recombinant Rat CCN family member 4 (Ccn4), Biotinylated	E.coli	N-terminal MBP-tagged and C-terminal 6xHis-Avi-tagged	CSB-EP857523RA-B
CCR7	Recombinant Human C-C chemokine receptor type 7 (CCR7), partial	E.coli	N-terminal 10xHis-tagged and C-terminal Myc-tagged	CSB-EP004846HU1
CCR7	Recombinant Human C-C chemokine receptor type 7 (CCR7)	in vitro E.coli expression system	N-terminal 6xHis-tagged	CSB-CF004846HUa0
CD274	Recombinant Human Programmed cell death 1 ligand 1 (CD274), partial	E.coli	N-terminal 10xHis-tagged and C-terminal Myc-tagged	CSB-EP878942HU2
CD274	Recombinant Human Programmed cell death 1 ligand 1 (CD274), partial (Active)	Mammalian cell	C-terminal hFc1-tagged	CSB-MP878942HU1
CD274	Recombinant Dog CD274 molecule (CD274), partial	Mammalian cell	C-terminal 10xHis-tagged	CSB-MP5015DO1
CD274	Recombinant Human Programmed cell death 1 ligand 1 (CD274), partial ( (K136A))	E.coli	N-terminal 6xHis-tagged	CSB-EP878942HU1(M)
CD274	Recombinant Human Programmed cell death 1 ligand 1 (CD274) (K136A), partial	Yeast	C-terminal 6xHis-tagged	CSB-YP878942HU1(M)c7
CD274	Recombinant Human Programmed cell death 1 ligand 1 (CD274), partial	Mammalian cell	C-terminal 10xHis-tagged	CSB-MP878942HU1d7
Cldn1	Recombinant Rat Claudin-1 (Cldn1), partial	E.coli	N-terminal 10xHis-tagged and C-terminal Myc-tagged	CSB-EP005490RA1
CLDN1	Recombinant Human Claudin-1 (CLDN1)	in vitro E.coli expression system	N-terminal 10xHis-tagged	CSB-CF005490HU
CLDN1	Recombinant Human Claudin-1 (CLDN1)-VLPs	Mammalian cell	C-terminal 10xHis-tagged (This tag can be tested only under denaturing conditions)	CSB-MP005490HU

● 抗體

CD274 Recombinant Monoclonal Antibody
CSB-RA878942MA1HU

Tested Applications:
ELISA, IHC, FC

CD38 Recombinant Monoclonal Antibody
CSB-RA796695A0HU

Tested Applications:
ELISA, WB, IHC

ITGAL Recombinant Monoclonal Antibody
CSB-RA787996A0HU

Tested Applications:
ELISA, WB, IF

PCSK9 Recombinant Monoclonal Antibody
CSB-RA259644A0HU

Tested Applications:
ELISA, WB, IHC

- 重組抗體

Target	Product Name	Species Reactivity	Tested Applications	Code
CD274	CD274 Recombinant Monoclonal Antibody	Human	ELISA, WB, IHC	CSB-RA977797A0HU
CD274	CD274 Recombinant Monoclonal Antibody	Human	ELISA, IHC, FC	CSB-RA878942MA1HU
CD38	CD38 Recombinant Monoclonal Antibody	Human	ELISA, WB, IHC	CSB-RA796695A0HU
CD40	CD40 Recombinant Monoclonal Antibody	Human	ELISA, IF, FC	CSB-RA004936MA1HU
GCGR	GCGR Recombinant Monoclonal Antibody	Human	ELISA	CSB-RA009316A1HU
IL17A	IL17A Recombinant Monoclonal Antibody	Human	ELISA	CSB-RA624104MA1HU
IL23A	IL23A Recombinant Monoclonal Antibody	Human	ELISA, WB	CSB-RA251959A0HU
IL6	IL6 Recombinant Monoclonal Antibody	Human	ELISA	CSB-RA011664MA1HU
ITGAL	ITGAL Recombinant Monoclonal Antibody	Human	ELISA, WB, IF	CSB-RA787996A0HU
MS4A1	CD20 Recombinant Monoclonal Antibody	Human	ELISA	CSB-RA015007A0HU
MS4A1	MS4A1 Recombinant Monoclonal Antibody	Human	ELISA	CSB-RA015007A1HU
MS4A1	MS4A1 Recombinant Monoclonal Antibody	Human, Dog, Macaca fascicularis	ELISA	CSB-RA015007MA3HU
MS4A1	MS4A1 Recombinant Monoclonal Antibody	Human	ELISA, IHC, IF	CSB-RA015007MA4HU
PCSK9	PCSK9 Recombinant Monoclonal Antibody	Human	ELISA, WB, IHC	CSB-RA259644A0HU
SELPLG	SELPLG Recombinant Monoclonal Antibody	Human	ELISA, IHC	CSB-RA567010A0HU

- 單克隆抗體

Target	Product Name	Species Reactivity	Tested Applications	Code
CD2	CD2 Monoclonal Antibody	Human,Mouse,Rat	ELISA, IHC	CSB-MA000227
CD2	CD2 Monoclonal Antibody,FITC Conjugated	Human	ELISA, IF, FC	CSB-MA785710
CD38	CD38 Monoclonal Antibody	Human	ELISA, IHC	CSB-MA785760
IL1B	IL1B Monoclonal Antibody	Human	ELISA	CSB-MA065741A0m
IL1B	IL1B Monoclonal Antibody	Human	ELISA, WB, IHC, ICC	CSB-MA010884
IL6	IL6 Monoclonal Antibody	Human	ELISA, WB, IHC	CSB-MA067571A0m
ITGAL	ITGAL Monoclonal Antibody,FITC Conjugated	Human	ELISA, IF, FC	CSB-MA120024
ITGAL	ITGAL Monoclonal Antibody,PE Conjugated	Human	ELISA, IF, FC	CSB-MA897229
MS4A1	MS4A1 Monoclonal Antibody	Human,Mouse,Rat	ELISA, IHC	CSB-MA000204
MS4A1	MS4A1 Monoclonal Antibody	Human	ELISA, IHC	CSB-MA996413
TNF	TNF Monoclonal Antibody	Bovine	ELISA	CSB-MA084771A0m
TNF	TNF Monoclonal Antibody	Human,Mouse,Rat	ELISA, WB, IHC	CSB-MA080271
TNF	TNF Monoclonal Antibody	Human,Mouse,Rat	ELISA, WB, IHC	CSB-MA080272

- 多克隆抗體

Target	Product Name	Species Reactivity	Tested Applications	Code
CD2	CD2 Antibody	Human	ELISA, IHC, IF	CSB-PA004894LA01HU
CD2	CD2 Antibody	Human	ELISA, WB	CSB-PA005194
CD2	CD2 Antibody	Human	ELISA, WB	CSB-PA004894GA01HU
CD274	CD274 Antibody	Human	ELISA, IHC	CSB-PA878942ESR1HU
CD274	CD274 Antibody	Human, Mouse, Rat	ELISA, WB, IHC	CSB-PA004911GA01HU
CD28	CD28 Antibody	Human	ELISA, WB	CSB-PA005199
CD28	Phospho-CD28 (Y218) Antibody	Human, Mouse	ELISA, WB	CSB-PA070302
CD38	CD38 Antibody	Human	ELISA, IHC	CSB-PA004929ESR1HU
CD38	CD38 Antibody	Human	ELISA, WB	CSB-PA005628
CD38	CD38 Antibody	Human	ELISA, WB	CSB-PA134528
CD38	CD38 Antibody	Human	ELISA, WB, IHC	CSB-PA926996
CD3D	CD3D Antibody	Human	ELISA, IHC	CSB-PA004930ESR1HU
CD3D	CD3D Antibody	Human	ELISA, WB	CSB-PA001452
CD3D	CD3D Antibody	Human	ELISA, WB	CSB-PA005826
CD3D	CD3D Antibody	Human, Mouse, Rat	ELISA, WB, IF	CSB-PA004930GA01HU
CD40	CD40 Antibody	Human, Mouse	ELISA, WB, IF	CSB-PA004936LA01HU
CD40	CD40 Antibody	Human, Mouse, Monkey	ELISA, WB, IHC	CSB-PA001455
CD40	Phospho-CD40 (T254) Antibody	Human, Mouse	ELISA, WB	CSB-PA080031
CD40	CD40 Antibody	Human	ELISA, WB	CSB-PA004936GA01HU
CD40	CD40 Antibody	Human, Mouse	ELISA, IHC	CSB-PA215588

● ELISA試劑盒

Rat Transforming Growth Factor β1(TGF-β1) ELISA kit
CSB-E04727r

Mouse monocyte chemotactic protein 1/monocyte chemotactic and activating factor,MCP-1/MCAF ELISA kit
CSB-E07430m

Mouse Interleukin 13,IL-13 ELISA Kit
CSB-E04602m

Human monocyte chemotactic protein 1/monocyte chemotactic and activating factor,MCP-1/MCAF ELISA kit
CSB-E04655h

Target	Product Name	Sample Types	Detection Range	Code
CCL2	Human high sensitivity C-C motif chemokine 2 (CCL2) ELISA kit	serum, plasma, tissue homogenates	6.25 pg/mL-400 pg/mL	CSB-EQ004783HU
TGFB1	Rat Transforming Growth Factor β1(TGF-β1) ELISA kit	serum, plasma, cell culture supernates, tissue homogenates	6.25 pg/mL-400 pg/mL	CSB-E04727r
TGFB1	Human Transforming Growth factor β1,TGF-β1 ELISA kit	serum, plasma, cell culture supernates	0.78 ng/mL-50 ng/mL	CSB-E04725h
TGFB1	Mouse Transforming Growth factor β1,TGF-β1 ELISA kit	serum, plasma, cell culture supernates	0.78 ng/mL-50 ng/mL	CSB-E04726m
CCL2	Mouse monocyte chemotactic protein 1/monocyte chemotactic and activating factor,MCP-1/MCAF ELISA kit	serum, plasma, tissue homogenates	78 pg/mL-5000 pg/mL	CSB-E07430m
CCL2	Rat monocyte chemotactic protein 1/monocyte chemotactic and activating factor,MCP-1/MCAF ELISA kit	serum, plasma, tissue homogenates	31.25 pg/mL-2000 pg/mL	CSB-E07429r
IL13	Mouse Interleukin 13,IL-13 ELISA Kit	serum, plasma, tissue homogenates	78 pg/ml - 5000 pg/ml	CSB-E04602m
CCL2	Human monocyte chemotactic protein 1/monocyte chemotactic and activating factor,MCP-1/MCAF ELISA kit	serum, plasma, urine, saliva, cerebrospinal fluid (CSF), ascitic fluid, cell culture supernates, tis	6.25 pg/ml-400 pg/ml.	CSB-E04655h
CD274	Human Programmed Death Ligand-1(PD-L1/CD274) ELISA Kit	serum, plasma, tissue homogenates, cell lysates	15.6 pg/mL-1000 pg/mL	CSB-E13644h
IL13	Rat Interleukin 13,IL-13 ELISA KIT	serum, plasma, cell culture supernates, tissue homogenates	7.8 pg/mL-500 pg/mL	CSB-E07454r
TGFB1	Chicken Transforming Growth factor β1,TGF-β1 ELISA kit	serum, plasma, tissue homogenates	0.312 ng/mL-20 ng/mL	CSB-E09875Ch
IL13	Human Interleukin 13,IL-13 ELISA KIT	serum, plasma, tissue homogenates	0.39 ng/ml-25 ng/ml.	CSB-E04601h
CLDN1	Human Claudin-1(CLDN1) ELISA kit	serum, plasma, tissue homogenates, cell lysates	15.6 pg/mL-1000 pg/mL	CSB-EL005490HU
TGFB1	Pig transforming growth factor β1(TGF-β1)ELISA Kit	serum, plasma	0.78 ng/mL-50 ng/mL	CSB-E06843p
IL11	Mouse Interleukin 11,IL-11 ELISA KIT	serum, plasma, tissue homogenates	78 pg/ml - 5000 pg/ml	CSB-E04597m
CCL2	Rabbit monocyte chemotactic protein 1/monocyte chemotactic and activating factor,MCP1/MCAF ELISA kit	serum, plasma, tissue homogenates	23.5 pg/mL-1500 pg/mL	CSB-E06921Rb
ENO1	Human Alpha-enolase (ENO1/ENO1L1/MBPB1/MPB1) ELISA kit	serum, tissue homogenates, plasma	0.312 ng/mL-20 ng/mL	CSB-E17177h
CXCR4	Rat CXC-chemokine receptor 4(CXCR4)ELISA Kit	serum, plasma, cell culture supernates, tissue homogenates	0.156 ng/mL-10 ng/mL	CSB-E12703r
CXCR4	Human CXC-chemokine receptor 4(CXCR4)ELISA Kit	serum, plasma, tissue homogenates	15.6 pg/mL-1000 pg/mL	CSB-E12825h
CCL2	Pig monocyte chemotactic protein 1,MCP1 ELISA Kit	serum, plasma	31.25 pg/mL-2000 pg/mL	CSB-E13178p