Cystic Fibrosis Article

Rogue Protein May Be Key to Cystic Fibrosis

Fri Jul 7, 7:09 PM ET

FRIDAY, July 7 (HealthDay News) -- U.S. researchers say they've identified and controlled an overactive protein that may be a key player in cystic fibrosis (CF).

CF is a genetic disorder that interferes with the body's ability to transport chloride in and out of cells.

According to researchers at Johns Hopkins Children's Center, Baltimore, the newly identified protein, called VCP/pr97, kills a chloride transporter in the cells of most CF patients. This inability to transport chloride results in a dangerous buildup of thick, sticky mucous in several organs, including the pancreas and lungs. That can lead to the malnutrition, chronic lung infection and lung damage so often seen in CF patients.

In this test-tube study, the researchers used a tool called RNA interference to successfully intercept signals sent out by VCP/pr97. In doing so, they were able to prevent cell damage caused by the protein. The findings showed promise in restoring cells to normal status in CF patients, the team said.

"The hope is that these findings will be used to design therapies and drugs that go beyond symptoms management and actually restore normal cell function to prevent CF," senior investigator Dr. Pamela Zeitlin, a pulmonologist at Johns Hopkins Children's Center, said in a prepared statement.

However, it will take years before any treatments based on this research are developed or tested on animals or humans, she noted.

The study was published in the June 23 issue of the Journal of Biological Chemistry.

Crank caller

We have had a slightly disqueting morning here at work...last week, we received several prank calls who we had believed came from a friend of the store. He has called us before, joking about things, but its usually about product, not at all like these calls.

Amber had the first call, in which in a quiet, sickly-sounding voice he asked her what color her bra was. She responded, "excuse me?". She talked to him a moment more, and thought it was Sam. Asked what he was doing, he responded working at home (which Sam does). She said he must be pretty bored, and he responded that he was, that he was just laying around naked doing nothing. Did that bother her? She said no, she wasn't at his house. He called back two more times that day. Another thing that made it odd is that usually when we tell Sam we need to call him back, he says fine. This guy wanted to call us back in a few minutes.

I received another call, in which he asked again what color bra I was wearing. At first I couldn't understand him, he was so difficult to hear. When I did, I was disgusted and hung up immediately. I told Jen about it, and she mentioned the other calls and how they thought it was Sam. Oh, okay. Thought nothing further of it.

Till today. He called again, and got Kristin. She again thought it was Sam. Asked him what he was doing today, was he going to stop by? (Sam and Kristin do things more often together than the rest of us) She commented that she would be going on break around 2:30pm. At some later point, she started to get suspicious that it wasn't Sam. Asked him things like what was the color of her hair, her last name, what was her tattoo of. He got them all wrong. After the call she told me all of this, and we were definitely concerned. So it also turns out that he had called later last week and talked to her, and she was going on break, asked him to call her back on her cell phone. He said his phone was in his car, could she give the number to him so he woudn't have to run out to the car. She never did get any sort of call, prank or otherwise. She went into the office after we talked to call Sam, and he said it definitely wasn't him, and that he had been out of town even. She tried *69ing the call, and couldn't get the number.

By now we were definitely concerned and talked this over more with Amber when she came in for work. Since Chad was coming in to fix the hanger bars, I called and asked him if he could be here around 2:30, and he said he thought we should call security. So we did, and an ATL cop came up and we discussed everything with him. He told us to keep track of the times and frequencies of the calls, and that the even though the call was untraceable through *69 if we called the phone company they may be able to find out more for us.

Hopefully this guy won't show up at 2:30. How would we even really know if he did? So we are all decidely a little weirded out by all of this....and I am really glad that Chad is coming in soon, as none of our male staffers are in today. Will keep you posted!