Johns Hopkins Medicine

The Peripheral Nerve Center at Johns Hopkins has assembled a group of specialists that provide expertise in most nerve disorders, including diagnosis and both medical and surgical treatments. The Center was founded to provide the highest quality care for peripheral nerve diseases, including surgical options, and to generate new understanding of the diagnosis and treatment of nerve diseases through research. The goals of the Peripheral Nerve Center at Johns Hopkins University are to develop new therapies that will reverse peripheral neuropathies by preventing axonal degeneration and restoring function to patients with sensory neuropathies; develop novel diagnostic strategies, or biomarkers, to more accurately and sensitively diagnose, track and monitor therapy for sensory neuropathies; a special emphasis on identifying underlying etiologies of painful peripheral neuropathies that are largely classified as "idiopathic"; and provide a collaborative training environment for neuroscientists committed to neuropathy research to facilitate care development and skill acquisition.

To these ends, the Foundation has dedicated funds to assist the Center with the expansion of a variety of initiatives. In order to speed the pace at which clinical trials may be conducted, the Foundation has promised funds to develop dedicated clinical trial infrastructure specific for peripheral neuropathy research. The foundation has supported the screening and animal modeling of existing drugs for treatment of peripheral neuropathy and the regeneration of lost nerve cells, and the examination of the role of supporting cell changes in chronic peripheral neuropathies. Based on the research results, additional funding has been provided to Johns Hopkins to continue their research on the four compounds identified in their initial studies.

Update on FPN funded research at Johns Hopkins:

Peripheral nerve regeneration in humans is suboptimal despite many years of research on various treatments demonstrating remarkable efficacy in animal models. This is partly due to the fact that human nerves are much longer and even though the peripheral axons can regenerate, Schwann cells, their glial cell partners in peripheral nerves, atrophy and do support regeneration with chronic denervation (reviewed in (Hoke, 2006)).

This proposal aims to build on our previous cycle of funding and evaluate a group of genes we have identified in denervated Schwann cells. We will examine their relevance to Schwann cell atrophy and lack of support for PNS regeneration. We will utilize the knowledge gained from these studies in parallel with other projects in the lab that identified small molecule drugs that enhance the rate of axon outgrowth in sensory neurons. The primary focus of the lab is to enhance peripheral nerve regeneration through both increasing the intrinsic capacity of axons to regenerate and the extrinsic influence of Schwann cells.

B. Specific Aims:

Specific aim of the proposal is:

To validate the role of various transcription factors, cell cycle genes and cell death pathway genes in maintenance of Schwann cells during chronic denervation. Specifically we will test whether manipulation of these genes (upregulation or downregulation) will have an impact on Schwann cell apoptosis in vitro.

Hypotheses to be tested:

• Downregulation of cell death pathway genes upregulated during late stages of chronic denervation will prevent Schwann cell atrophy and death

• Upregulation of transcription factors downregulated during late stages of chronic denervation will prevent Schwann cell atrophy and death

Published updates: ILARJ; ANA2014

FPN Peripheral Neuropathy Research Registry

Collaborative efforts between our partners and other institutions help us work toward a greater understanding of the cause and progression of PN.  The Foundation is proud to provide the financial investments required to lead new medical innovations into the future. In addtion to other funding provided to Johns Hopkins, The Foundation has awarded a grant for them to participate in FPN's groundbreaking new patient registry for people with painful and non-painful neuropathies.

Learn more about the Peripheral Neuropathy Research Registry.