Medicine makes a break­through in cys­tic fi­bro­sis

Im­mune cells could be key to treat­ment

The Press and Journal (Aberdeen) - - NEWS - BY HI­LARY DUNCANSON

Fresh in­sights into how cys­tic fi­bro­sis af­fects im­mune cells could pave the way for new treat­ments for the con­di­tion, re­searchers be­lieve.

Sci­en­tists have dis­cov­ered that cells in pa­tients with cys­tic fi­bro­sis that nor­mally de­fend against in­fec­tion can also per­pet­u­ate dam­age to the lungs.

Drugs that tar­get th­ese cells could help to stem pro­gres­sion of the disease, they claim.

A team at the Uni­ver­sity of Ed­in­burgh, with re­searchers in the US and Ire­land, fo­cused on im­mune cells known as neu- trophils, part of the body’s first line of de­fence against in­fec­tions.

Once an in­fec­tion has been cleared, neu­trophils are usu­ally pro­grammed to die off qui­etly, so that they do not mis­tak­enly cause dam­age to healthy tis­sues.

How­ever, in pa­tients with cys­tic fi­bro­sis, neu­trophils sur­vive longer than they are sup­posed to and are a key con­trib­u­tor to the lung dam­age asso- ciated with the con­di­tion, ex­perts said.

The team dis­cov­ered that neu­trophils from cys­tic fi­bro­sis pa­tients are more re­sis­tant to the usual mech­a­nism of cell death – a process called apop­to­sis – with their abil­ity to sur­vive longer di­rectly re­lated to the un­der­ly­ing ge­netic mu­ta­tion that causes cys­tic fi­bro­sis.

In­stead, the cells die by a dif­fer­ent process, which causes them to dis­in­te­grate and ex­pel their dam­ag­ing con­tents into the sur­round­ing area of the lung, ex­perts said.

This process pro­motes in­flam­ma­tion and may there­fore pro­mote dam- age to healthy tis­sues in the lung.

Re­searchers said they were able to block this process with a drug that en­cour­ages neu­trophils to die by apop­to­sis.

Dr Robert Gray, of the Med­i­cal Re­search Coun­cil Cen­tre for In­flam­ma­tion Re­search at Ed­in­burgh Uni­ver­sity, said: “Ther­a­pies tar­get­ing in­flam­ma­tion are not read­ily avail­able but are needed for the treat­ment of cys­tic fi­bro­sis.

“This work, although at an early stage, will help in the de­vel­op­ment of new anti-in­flam­ma­tory treat­ments for this de­bil­i­tat­ing con­di­tion.”

“This work, still at an early stage, will help in de­vel­op­ment”

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