The 9-year-old girl is just happy to compete in the sixth annual PossAbilities Triathlon today at Loma Linda University.

Abbey - who has a rare form of muscular dystrophy - will compete against other able-bodied athletes in her age group.

"First, we run, then we bike, then we swim," said Abbey, the Muscular Dystrophy Association's 2008 National Goodwill Ambassador. "It's really fun."

When she was 2 years old, Abbey was diagnosed with a form of Charcot- Marie-Tooth disease, characterized by lack of balance and coordination as well as muscle weakness.

Abbey walks with the aid of leg braces.

But the third-grader at Montessori in Redlands does not feel sorry for herself.

Abbey loves to read, sing, play the piano and visit libraries, bookstores and animal parks. When she grows up, Abbey wants to be a veterinarian.

As ambassador, she travels through the United States and represents families that are affected by neuromuscular diseases and participates in Muscular Dystrophy Association events.

Today, Abbey will be competing in her third triathlon.

"She was born with a lot of determination," said Wendi Umali, Abbey's mother. "She doesn't let anything get her down. Anything she wants to do, she just goes after it."

To cheer her on
The sixth annual PossAbilities Triathlon is at 7 a.m. today at Drayson Center on the Loma Linda University campus, 25040 Stewart St.

Using human embryonic stem cells, the Menlo Park company has developed a therapy that enables paralyzed rats to walk and that it claims shows no dangerous side effects in experiments with about 2,000 animals.
Others also are studying such cells for medical uses, including Stanford University scientists, who last week said they had used them to help stroke-disabled lab rats walk better. But none are as close to seeking permission for human tests as Geron, whose treatment is for spinal injuries.

For its application requesting regulatory approval from the U.S. Food and Drug Administration, the public company has gathered 25,000 pages of data - far more than normal for such requests, Geron Chief Executive Dr. Thomas Okarma said. He told analysts recently that Geron would submit it to the FDA during the first part of this year. But he declined to reveal the actual filing date, he said, "to minimize any kind of pressure on the agency."

Yet Geron's bid isn't certain.

Although the FDA would not comment on Geron's application, President Bush objects to most research with embryonic stem cells, which come from discarded embryos. Moreover, his administration has become intrigued with recent studies showing skin cells can be manipulated to have embryonic-like properties without harming an embryo.

Read the rest of One Big Step for Geron.

Scholz J, Woolf CJ.

Neural Plasticity Research Group, Department of Anesthesia and Critical Care, Massachusetts General Hospital and Harvard Medical School, Charlestown, Massachusetts 02129, USA.

Nociceptive pain results from the detection of intense or noxious stimuli by specialized high-threshold sensory neurons (nociceptors), a transfer of action potentials to the spinal cord, and onward transmission of the warning signal to the brain. In contrast, clinical pain such as pain after nerve injury (neuropathic pain) is characterized by pain in the absence of a stimulus and reduced nociceptive thresholds so that normally innocuous stimuli produce pain. The development of neuropathic pain involves not only neuronal pathways, but also Schwann cells, satellite cells in the dorsal root ganglia, components of the peripheral immune system, spinal microglia and astrocytes. As we increasingly appreciate that neuropathic pain has many features of a neuroimmune disorder, immunosuppression and blockade of the reciprocal signaling pathways between neuronal and non-neuronal cells offer new opportunities for disease modification and more successful management of pain.

Developmental loss of NT-3 in vivo results in reduced levels of myelin-specific proteins, a reduced extent of myelination and increased apoptosis of Schwann cells.

Woolley AG, Tait KJ, Hurren BJ, Fisher L, Sheard PW, Duxson MJ.

Department of Pathology, Dunedin School of Medicine, University of Otago, Dunedin, New Zealand.

This work investigates the role of NT-3 in peripheral myelination. Recent articles, based in vitro, propose that NT-3 acting through its high-affinity receptor TrkC may act to inhibit myelin formation by enhancing Schwann cell motility and/or migration. Here, we investigate this hypothesis in vivo by examining myelination formation in NT-3 mutant mice. On the day of birth, soon after the onset of myelination, axons showed normal ensheathment by Schwann cells, no change in the proportion of axons which had begun to myelinate, and no change in either myelin thickness or number of myelin lamellae. However in postnatal day 21 mice, when myelination is substantially complete, we observed an unexpected reduction in mRNA and protein levels for MAG and P(0), and in myelin thickness. This is the opposite result to that predicted from previous in vitro studies, where removal of an inhibitory NT-3 signal would have been expected to enhance myelination. These results suggest that, in vivo, the importance of NT-3 as a major support factor for Schwann cells (Meier et al., (1999) J Neurosci 19:3847-3859) over-rides its potential role as an myelin inhibitor, with the net effect that loss of NT-3 results in degradation of Schwann cell functions, including myelination. In support of this idea, Schwann cells of NT-3 null mutants showed increased expression of activated caspase-3. Finally, we observed significant reduction in width of the Schwann cell periaxonal collar in NT-3 mutant animals suggesting that loss of NT-3 and resulting reduction in MAG levels may alter signaling at the axon-glial interface.

In the current issue of Biomaterials, scientists from Wake Forest University School of Medicine reported that in animal studies the protein keratin was able to speed up nerve regeneration and improve nerve function compared to current treatment options.

“We found that the nerve repair happened more quickly and consistently, and that functional recovery was higher,” said Mark Van Dyke, Ph.D., senior author and an assistant professor of regenerative medicine. “The fact that we were able to accomplish this with gels made from keratin is pretty remarkable.”

Grafting a nerve from another part of the body is usually the most effective option, but it creates another injury site and isn’t possible in all patients. The tubes, known as nerve guidance conduits, cannot be used in gaps longer than three or four centimeters. In addition, nerve regeneration with this method is not always successful. For example, after about age 17, nerves don’t regenerate as well.

Laboratory scientists have tried placing natural materials, such as collagen, into the conduits to promote nerve regeneration. Van Dyke’s team was the first to use keratin, which is believed to contain molecules that regulate cell behavior.

The scientists collected human hair from a local barber shop and chemically processed it to remove the keratin. They purified the keratin protein and used it to form gels that were then used to fill the nerve guidance conduits. They studied how keratin affects the activity of Schwann cells, which play a vital role in nerve regeneration. These cells produce signals that tell nerve cells to begin regenerating and “remodel” the blood clot that has formed so that nerve cells can grow across it.

“By using keratin to activate these cells, we’re trying to tap into the natural healing cascade,” said Van Dyke. “We believe that keratin helps amp up Schwann cell activity and give the nerve regeneration process a head start.”

The laboratory studies showed that keratin activated Schwann cells and increased their proliferation and migration. Next, the scientists used a keratin-filled tube to attempt to repair a 4 millimeter nerve gap in mice -- a fairly significant gap considering the size of the animal.

The results from these animals were compared with animals treated with an empty nerve guidance conduit and with animals treated with a nerve graft.

After six weeks, 100 percent of the animals in the keratin and nerve graft groups showed visible nerve regeneration across the gap, compared to only 50 percent who got the empty conduit. The speed of repair was best in the keratin group.

The scientists then tested the function of the regenerated nerve. The speed of nerve impulses was best in the keratin group. The amount of signal that got through the nerve was better in the keratin group than in the empty tube group. The study was recently highlighted in the journal Science.

“The results suggest that a conduit filler derived from hair keratins can promote an outcome comparable to a grafted nerve,” said Van Dyke.

In the study, the nerve function did not translate into recovery of muscle function, but the scientists suspect they may have tested too early, before the nerve had time to regenerate to the muscle. It is known that muscle function recovery lags behind nerve recovery. Future studies will focus on regeneration across larger gaps and will test whether nerve regeneration results in a return of muscle function.

Abbey is the first MDA national goodwill ambassador to hail from California in the program’s 55-year history. She and her family will travel the country throughout the year, representing families affected by neuromuscular diseases and served by MDA, participating in special events and meetings of national MDA sponsors and speaking with the media.

“Abbey is precocious and charismatic,” MDA National Chairman Jerry Lewis said. “I’m delighted to have such a wonderful young lady and her family spread the word about MDA’s commitment to finding treatments for neuromuscular diseases.”

Abbey is the only child of Joel and Wendi Umali. Her father is a dentist and her mother is a physical therapist.

Abbey has congenital hypomyelinating neuropathy, a form of Charcot-Marie-Tooth disease characterized by lack of coordination and balance, along with muscle weakness. She walks with the aid of leg braces.

A third-grader at Montessori School in Redlands, she loves to read, sing, swim, ride her bike, play the piano, act in plays and spend time with her friends. She also likes visiting the library, bookstores and animal parks. When she grows up, Abbey wants to be a veterinarian.

Television viewers worldwide met Abbey and her family when they were profiled and interviewed live on the 2006 and 2007 MDA Telethons.

The Umalis already take part in many local events for MDA because Abbey is serving her second term as MDA’s Goodwill Ambassador for California.

In her new role, she succeeds 14-year-old Luke Christie of Due West, S.C., who served two terms as National Goodwill Ambassador.

MDA is a voluntary health agency working to defeat more than 40 neuromuscular diseases through programs of worldwide research, comprehensive services and far-reaching professional and public health education.

Abbey receives services at Loma Linda University Medical Center, site of one of MDA’s 225 hospital-affiliated clinics nationwide.

The Association’s programs are funded almost entirely by individual private contributors.

"We focused on the vascular and neurochemical environment within the tube," said Tomoyuki Yamakawa, MD, the study's lead author. "We thought that BMCs containing adult stem cells, with the potential to differentiate into bone, cartilage, fat, muscle, or neuronal cells, could survive by obtaining oxygen and nutrients, with the result that rates of cell differentiation and regeneration would improve."

Nourished with bioengineered additives, such as growth factors and cell adhesion molecules, the BMCs after 24 weeks differentiated into cells with characteristics of Schwann cells -- a variety of neural cell that provides the insulating myelin around the axons of peripheral nerve cells. The new cells successfully regenerated axons and extended their growth farther across nerve cell gaps toward damaged nerve stumps, with healthier vascularity.

"The differentiated cells, similar to Schwann cells, contributed significantly to the promotion of axon regeneration through the tube," explained Yamakawa. "This success may be a further step in developing artificial nerves."

Read more of Bone Marrow Cell Transplants Help Nerve Regeneration

Protein Linked to 3 Nerve Ailments

IN two papers representing the work of 19 researchers, scientists reported last week that they had seen, at a molecular level, the damage to an important protein that is the cause of three genetic nerve disorders. Dr. Thomas Bird, a professor at the University of Washington and chief of neurology at the Veterans Affairs hospital in Seattle, who is not associated with the groups who made the reports, said that the papers are examples of where medicine has arrived: at the molecular detail of human disease.

Read more of Protein Linked to 3 Nerve Ailments

Its purpose: changing the way medicine works.

''The future of medicine depends entirely on projects from the field of genomics,'' or the study of all the genes in humans, medical school dean Dr. Pascal Goldschmidt said at Tuesday's opening.

The University of Miami institute is only the second of its kind in the United States. The Broad Institute of Harvard University and Massachusetts Institute of Technology was founded in 2003. Genomic research is part of the focus at Scripps Institute at Florida Atlantic University in Boca Raton, where 230 researchers are looking at discovering new drugs.

The UM genomics institute will focus on the genetic origins of multiple sclerosis, age-related macular degeneration, amyotrophic lateral sclerosis (Lou Gehrig's disease), tuberculosis and Charcot-Marie-Tooth disease, as well as cardiovascular disease, neurodevelopmental disorders and cancer.

Read the rest of University of Miami center seeks diseases' origins

The Salk study, led by Samuel Pfaff, Ph.D, a professor in the Gene Expression Laboratory, identifies a mutation they christened Magellan, after the Portuguese mariner whose ship Victoria was first to circumnavigate the globe. The Magellan mutation occurs in a gene that normally pilots motor neurons on the correct course employing a newly discovered mechanism, their results demonstrate.

Read the rest of Key Nerve Navigation Pathway Identified

Anand P, Kunnumakkara AB, Newman RA, Aggarwal BB.

Cytokine Research Laboratory and Pharmaceutical Development Center, Department of Experimental Therapeutics, The University of Texas M. D. Anderson Cancer Center, Houston, Texas 77030

Curcumin, a polyphenolic compound derived from dietary spice turmeric, possesses diverse pharmacologic effects including anti-inflammatory, antioxidant, antiproliferative and antiangiogenic activities. Phase I clinical trials have shown that curcumin is safe even at high doses (12 g/day) in humans but exhibit poor bioavailability. Major reasons contributing to the low plasma and tissue levels of curcumin appear to be due to poor absorption, rapid metabolism, and rapid systemic elimination.

To improve the bioavailability of curcumin, numerous approaches have been undertaken. These approaches involve, first, the use of adjuvant like piperine that interferes with glucuronidation; second, the use of liposomal curcumin; third, curcumin nanoparticles; fourth, the use of curcumin phospholipid complex; and fifth, the use of structural analogues of curcumin (e.g., EF-24). The latter has been reported to have a rapid absorption with a peak plasma half-life.

Despite the lower bioavailability, therapeutic efficacy of curcumin against various human diseases, including cancer, cardiovascular diseases, diabetes, arthritis, neurological diseases and Crohn's disease, has been documented. Enhanced bioavailability of curcumin in the near future is likely to bring this promising natural product to the forefront of therapeutic agents for treatment of human disease.

With $697,065 in grants from the New Jersey Commission on Spinal Cord Injury and the New Jersey Commission on Brain Injury Research, Haesun Kim of Teaneck, NJ, assistant professor of biological sciences at Rutgers University in Newark, is working on gaining a better understanding of those links.

Specifically, her work focuses on Schwann cells within the peripheral nervous system and their communication links with the axons they myelinate by enwrapping them in myelin. Axons are the long fibrous part of neurons that carry the nerve's electrical signals. A fatty substance, myelin covers those axons both to protect them and to provide a conduit for the fast conduction of electrical signals within the nervous system. Once that myelin is lost,the electrical signal breaks down and eventually the neuron dies -- like a cell phone that loses its signal.

"When Schwann cells are generated during development, axons send out signals to the Schwann cells and tell them, 'You are going to become myelin cells and you are going to myelinate me,'" explains Kim. "The Schwann cells in turn guide the axons to where they need to go and direct the axons to grow."

By pinpointing the sequence and nuances of the communication links involved in myelination, targeted genetic and pharmacological interventions possibly could be developed to restore the loss of myelin. Such an understanding additionally may allow for the effective transplanting of Schwann cells in the central nervous system to promote remyelination and the correction of neurological disorders at that level.

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Article adapted by Medical News Today from original press release.
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The New Jersey Commission on Spinal Cord Injury has provided $397,066 and the New Jersey Commission on Brain Injury Research $299,999 to support Kim's research.

Kim received her M.S. in biology from the University of Toledo, her Ph.D. in cell biology, neurobiology and anatomy from the University of Cincinnati, and performed her post-doctoral work at the Dana-Farber Cancer Institute at Harvard Medical School. She joined the Rutgers-Newark faculty in 2004.

Click here for more information on Dr. Kim's research.

Cleveland Clinic, Department of Neurosciences and Center for Neurological Restoration, 9500 Euclid Avenue, Cleveland, Ohio 44195, USA.

Peripheral nerve diseases, also known as peripheral neuropathies, affect 15-20 million of Americans and diabetic neuropathy is the most common condition. Currently, the treatment of peripheral neuropathies is more focused on managing pain rather than providing permissive conditions for regeneration. Despite advances in microsurgical techniques, including nerve grafting and reanastomosis, axonal regeneration after peripheral nerve injury remains suboptimal. Also, no satisfactory treatments are available at this time for peripheral neurodegeneration occurring in motor neuron diseases (MND), including amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA).

Peripheral nerves have the inherent capacity of regeneration. Gene therapy strategies focused on neuroprotection may help optimizing axonal regrowth. A better understanding of the cellular and molecular events involved in axonal degeneration and regeneration have helped researchers to identify targets for intervention. This review summarizes the current state on the clinical experience as well as gene therapy strategies for peripheral neuropathies, including MND, peripheral nerve injury, neuropathic pain, and diabetic neuropathy.

In addition to an all-you-can-eat fish and chicken dinner on Sunday, October 21st from 11:00 am - 2:00 pm, there will be a bake sale, a silent auction (which ends at 1:30), and a live auction (begins at 2:15). Location is St. Peter's Lutheran School, 7810 E. State Street, at the corner of State and Maysville.

Another chance to help Maddie is by getting your hair cut at Great Clips in Georgetown North on October 14th from 5:00 - 7:00 or until the last hair cut is complete. All proceeds will be donated to Maddie's fund.