Pulmonary fibrosis: Between a ROCK and a hard place

Pulmonary fibrosis is a scarring or thickening of the lungs that causes shortness of breath, a dry cough, fatigue, chest discomfort, weight loss, a decrease in the ability of the lungs to transmit oxygen to the blood stream, and, eventually, heart failure. Cells known as myofibroblasts normally secrete materials that are required for wound healing; once the wound has closed, the cells disappear. In pulmonary fibrosis, the myofibroblasts stick around, continuing to secrete wound healing factors that cause fibrosis in the lungs.

In this issue of the , Yong Zhou and colleagues at the University of Alabama at Birmingham identified a mechanosensitive cellular signaling pathway in myofibroblasts that is activated by the hardening of tissue that has become fibrotic.

Activation of this pathway promotes myofibroblast survival and prevents the normal disappearance of these cells after completion of wound healing. The pathway is dependent on a protein known as ROCK.

Zhou and colleagues found that a drug that inhibits ROCK, fasudil, attenuates the pro-survival pathway and causes myofibroblasts to die. Further, fasudil treatment protected mice from injury-induced .

These studies suggest that ROCK inhibitors could be used to treat . In a companion Attending Physician article, Dean Sheppard of the University of California, San Francisco, discusses the feasibility of using ROCK inhibitors in a clinical setting.

More information: Inhibition of mechanosensitive signaling in myofibroblasts ameliorates experimental pulmonary fibrosis, Journal of Clinical Investigation, 2013. doi:10.1172/JCI66700
ROCKing pulmonary fibrosis, Journal of Clinical Investigation, 2013. doi:10.1172/JCI68417

add to favorites email to friend print save as pdf

Related Stories

Scarred lungs leave trail of beta arrestins

Mar 28, 2011

Targeting a family of signaling proteins called beta arrestins may stop the life-threatening scarring and thickening of lungs associated with pulmonary fibrosis, reports a new Science study in mice.

Recommended for you

The impact of bacteria in our guts

Aug 22, 2014

The word metabolism gets tossed around a lot, but it means much more than whether you can go back to the buffet for seconds without worrying about your waistline. In fact, metabolism is the set of biochemical ...

Stem cell therapies hold promise, but obstacles remain

Aug 22, 2014

(Medical Xpress)—In an article appearing online today in the journal Science, a group of researchers, including University of Rochester neurologist Steve Goldman, M.D., Ph.D., review the potential and ch ...

New hope in fight against muscular dystrophy

Aug 22, 2014

Research at Stockholm's KTH Royal Institute of Technology offers hope to those who suffer from Duchenne muscular dystrophy, an incurable, debilitating disease that cuts young lives short.

Biologists reprogram skin cells to mimic rare disease

Aug 21, 2014

Johns Hopkins stem cell biologists have found a way to reprogram a patient's skin cells into cells that mimic and display many biological features of a rare genetic disorder called familial dysautonomia. ...

User comments