Paper highlight: Signaling hope for polycystic kidney disease

Sep 13, 2010

Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a common genetic disease that results in chronic kidney failure.

Although the genes responsible for ADPKD have been identified (PKD1, PKD2), relatively little is known about how mutations in these promote cyst growth molecularly.

In this paper, scientists at Children's Hospital in Boston, lead by Jordon Kreidberg, investigated the signaling pathways that go awry in the disease using mouse epithelial cells in which Pkd1 was genetically deleted.

They found that the protein c-Met was hyperactive in Pkd1-deficient cells, resulting in increased mTOR signaling, a pathway that had previously been linked to cyst formation. The increase in c-Met activity was related to sequestration of the protein c-Cbl in a cellular compartment known as the golgi, which increased c-Met protein stability.

In support of a critical role for c-Met activity in disease progression, pharmacological inhibition of c-Met decreased mTOR activity and blocked cyst formation in a of ADPKD, leading the authors to suggest that c-Met is a potential in patients with ADPKD.

Explore further: Cochrane Review of RDT for diagnosis of drug resistant TB

More information: Failure to ubiquitinate c-Met leads to hyperactivation of mTOR signaling in a mouse model of autosomal dominant polycystic kidney disease: www.jci.org/articles/view/4153… 07331a5bc3d8a1998c3f

Provided by Journal of Clinical Investigation

not rated yet
add to favorites email to friend print save as pdf

Related Stories

Experiments point to new treatments for PKD

Apr 02, 2008

A family of small molecules called CFTR inhibitors show promising effects in slowing the progression of polycystic kidney disease (PKD), the most common genetic disease of the kidneys, according to preliminary research reported ...

Recommended for you

Africans in New York complain of Ebola stigma

6 hours ago

Members of the west African community in New York complained Wednesday that their children were being bullied at school and businesses were losing money because of hysteria over Ebola.

Ebola expert says China at risk, seeks Japan aid

6 hours ago

A scientist who helped to discover the Ebola virus says he is concerned that the disease could spread to China given the large numbers of Chinese workers traveling to and from Africa.

User comments : 0

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.