Idiopathic Pulmonary Fibrosis (IPF)
- IPF is a condition which occurs an abnormal scarrings in the lungs by unknown cause that causes the lung to become thick and stiff.
As the disease progresses, the lung function exchanging oxygen decreases, eventually leading to death.
- A chronic progressive disease and It has a bad prognosis because the lung function impaired by it can not be recovered.
In general, about two-thirds of patients die within five years of diagnosis.
- It may occur in all age groups, but it usually occurs in middle-aged or older ages, and the incidence rates are rising in developed countries
where aging has progressed.
- Rare disease with orphan drug status.
- There is no cure. Only two drugs are in the market that just could slow down the progress of the disease.
- The Global market size is expected to grow from 0.7bn in 2017 to $ 2.9bn in 2021.
(Source: Datamonitor Healthcare ‘Idiopathic Pulmonary Fibrosis disease coverage 2017’)
- There are more than 100,000 patients in the United States and and between 30,000 and 40,000 new cases are occurring worldwide every
year. It has a incidence rate of 13-20 people per 100,000 people worldwide. (Source: NIH)
The Mechanism of ONG21001
- Prevention & Inhibition of fibrosis through EMT inhibition
If cells are injured by irritants
The injured cells release
Immunocytes move to the
The immunocytes release
substantce such as IL-1β and TGF-β.
IL-1β and TGF-β released from the immunocytes stimulate the epithelial cells and differentiate them into myofibroblasts.
The extracellular matrix(ECM) is generated by a series of actions involving the role of myofibroblasts, through which the wound is filled and reconstructed.
The wound is healed through this process, but if the inflammation is sustained, ECM is accumulated excessively that lead to fibrosis.
- ONG21001 prevent and suppress fibrosis through EMT inhibition
ONG21001 inhibits EMT induced by substances such as IL-1β and TGF-β
released from the immunocytes, thereby preventing excessive
accumulation of extracellular matrix in the wound.
This action of ONG21001 effectively prevent and suppress fibrosis caused by
- ONG21001 normalize the fibrotic cells through de-differentiation.
The myofibroblasts differentiated through EMT
continue to exist during the inflammation and
become a cause of progress of fibrosis.
ONG21001 selectively acts on the myofibroblasts
differentiated through EMT and get them
dedifferentiate into the original epithelial cells.
This action of ONG21001 normalize the fibrotic
cells and cure the fibrosis.
In vitro data
In vivo data
- Therapeutic efficacy test results in a mouse model of Bleomycin induced pulmonary fibrosis.
- ONG21001 has shown a significant therapeutic efficacy in the test and the results is better than one of the drug on the market.
- ONG21002 is an inhalation version of ONG21001 being developed for oral administration.
- ONG21010 is being developed for coadministration in the combination of ONG21001 & Pirfenidon.
- Pirfenidon is the best selling marketed IPF drug and it`s generics are on the market.
The development goal of ONG21001, ONG21002, ONG21010
- ONG21001/ONG21002/ONG21010 have a development goal of reaching higher therapeutic efficacy than the drugs on the market currently.