Autoimmune Disease Series--

Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive, and fatal form of interstitial pneumonia characterized by irreversible fibrotic remodeling of lung tissue, ultimately leading to respiratory failure. Its core pathological mechanisms include repeated injury to alveolar epithelial cells and abnormal repair processes, which drive the activation of fibroblasts into myofibroblasts. This results in excessive deposition of extracellular matrix, forming pulmonary scars.

Clinically, it presents with exertional dyspnea, dry cough, and fatigue, often accompanied by progressive decline in lung function. Approximately 50% of patients also have interstitial lung disease (ILD). IPF primarily affects individuals over 50 years old, with a median survival of only 2-5 years. Its prognosis is worse than many cancers, earning it the moniker "the cancer that isn't cancer."

Drug development for Idiopathic Pulmonary Fibrosis (IPF) focuses on core mechanisms like fibrosis, inflammation, and cellular repair, targeting key pathways including:

Transforming Growth Factor-beta (TGF-β) Pathway: The existing drug nintedanib delays lung function decline by inhibiting TGF-β downstream receptors (VEGFR, PDGFR, FGFR). Bristol Myers Squibb's BMS-986278 (Admilparant), a lysophosphatidic acid receptor 1 (LPAR1) antagonist, demonstrated a 54% reduction in the annual rate of forced vital capacity (FVC) decline in its Phase II study and is currently in Phase III trials.

Phosphodiesterase 4B (PDE4B): Boehringer Ingelheim's oral inhibitor nerandomilast significantly slowed FVC decline in the Phase III FIBRONEER-IPF trial with a manageable safety profile. It has been submitted for marketing approval in China and granted priority review.

Connective Tissue Growth Factor (CTGF): Pliant Therapeutics' bexotegrast (PLN-74809), which inhibits the binding of CTGF to TGF-β, showed reduced FVC decline in a Phase IIa study and is now advancing to Phase IIb/III trials.

Current IPF drug discovery is breaking new ground with multi-target approaches and novel mechanisms. Insilico Medicine leveraged AI to discover the TNIK kinase inhibitor INS018_055, which demonstrated superior anti-fibrotic effects to nintedanib in preclinical studies and synergistic effects when combined with pirfenidone. The National Institute of Biological Sciences, Beijing (NIBS), identified AREG protein as a key target, and its inhibitor has entered Phase I clinical trials. In the realm of cell therapies, Shanghai Jiao Tong University's autologous basal stem cell transplantation and Genmir Biotech's REGEND001 airway basal stem cell therapy have both shown positive outcomes, including improved lung function, in clinical trials. Furthermore, Korean research confirmed that ezetimibe inhibits fibroblast differentiation, and Chinese scientists identified the GOLM1-KLF4-NEAT1 signaling pathway as having potential value, offering new hope for IPF treatment.

Idiopathic Pulmonary Fibrosis Drug Targets in Development:

Idiopathic Pulmonary Fibrosis Drug Targets Related Products

● Target Proteins

CSB-MP5112MOW Activity Verified

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.

CSB-MP878942HU1 Activity Verified

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.

CSB-MP006254HU(F1) Activity Verified

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.

CSB-MP723415HU Activity Verified

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 Product 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

● Antibodies

CD274 Recombinant Monoclonal Antibody
CSB-RA878942MA1HU
CSB-RA878942MA1HU FC

Tested Applications:
ELISA, IHC, FC

CD38 Recombinant Monoclonal Antibody
CSB-RA796695A0HU
CSB-RA796695A0HU WB

Tested Applications:
ELISA, WB, IHC

ITGAL Recombinant Monoclonal Antibody
CSB-RA787996A0HU
CSB-RA787996A0HU IF

Tested Applications:
ELISA, WB, IF

PCSK9 Recombinant Monoclonal Antibody
CSB-RA259644A0HU
CSB-RA259644A0HU IHC

Tested Applications:
ELISA, WB, IHC

- Recombinant Antibody

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

- Monoclonal Antibody

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

- Polyclonal Antibody

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 Kits

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


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