Quest Magazine

Blocking a Protein Extends Survival in ALS Mice

Elevated levels of an immune-system protein called interleukin-1-beta (IL-1-beta) exacerbates a disease in mice that closely resembles human amyotrophic lateral sclerosis (ALS), and blocking this protein extends survival in these mice, a research team in Germany has found.

The study adds to the accumulating evidence that the immune system misbehaves in ALS and that altering its behavior could be a way to treat the disease.

Feds Both Seek and Give Advice Re Disability Issues

Two federal agencies are seeking to improve conditions for people with disabilities, in one case by strengthening federal hiring regulations, and in the other by making health care facilities more accessible to people with mobility problems.

Improving employment

The U.S. Department of Labor, through its Office of Federal Contract Compliance Programs (OFCCP), is inviting public comments on ways to strengthen regulations covering the hiring of people with disabilities by federal contractors.

August is SMA Awareness Month

August is National Spinal Muscular Atrophy Awareness Month, and research in this disease has never been more promising.

Major themes in spinal muscular atrophy (SMA) research include increasing cellular production of the needed SMN protein; inserting new genes for the needed SMN protein; stabilizing shorter SMN protein molecules produced by people with SMA; and strengthening the nerve-muscle connections (neuromuscular junctions) that are weakened in this disease.

Here are some recent news stories about SMA from the MDA website.

ALS-Causing TDP43 Overstays Its Welcome

At least some of the mutations in the TDP43 protein that are known to lead to ALS cause the TDP43 protein to be more stable than usual and change its interactions with other cellular proteins, say researchers at the University of California-San Diego. The changes are toxic ones.

Retiree with BMD is the Best at Beauty Pageants

Beauty pageants are not events that just happen by themselves, as Will Isgett can attest.

He’s one of 42 local executive directors of preliminary beauty pageants in South Carolina cities that culminate every year in the Miss South Carolina pageant. In 2010, the Miss South Carolina Board of Directors voted him the best of the bunch, for the pageant he directed in his hometown of Darlington.

Study to Probe Oxidative Stress in People With ALS

More than 400 people who recently received an ALS diagnosis are being sought for a large study of a cell-damaging phenomenon called "oxidative stress," common in ALS and other degenerative diseases.

Oxidative stress is a type of damage that results from high levels of toxic byproducts of energy production inside cells. These toxic chemicals — known as free radicals — normally are present, but when they're produced in excess or when cells become unable to detoxify them, they become dangerous.

‘Power for the Journey’ Gives Back to ALS Community

When you’re hoping to earn $14, but wind up making $20 instead, that’s good reason to be happy.

Marlene Zaleski and four of her friends found themselves in just such a situation, except the $14 was $14,000, and the final tally from their ALS fundraising run/walk in Cookeville, Tenn., on April 24 was not $20 but $20,118. All proceeds from the event went to MDA’s Augie’s Quest ALS research effort.

Utah Researchers Seek CMD Families

Families affected by either of two forms of congenital muscular dystrophy (CMD), known as Ullrich CMD and Bethlem myopathy, are invited to help with an MDA-supported data collection project at the University of Utah in Salt Lake City. The project's investigators are seeking to correlate genetic and clinical (symptom-related) information to improve understanding of these diseases and begin to develop treatments.

About Ullrich CMD and Bethlem myopathy

Nonwalkers With SMA At High Risk for Weight Gain

Children and adults with types 2 and 3 spinal muscular atrophy (SMA) who are no longer walking have an increased risk of being overnourished and overweight if they otherwise have relatively good motor function, a multicenter study shows.

The results emphasize the importance of a "dedicated and experienced nutritionist" in SMA medical management, the researchers say.

About the study

Antisense Treatment Restores Full-Length SMN in SMA Mice

Scientists have found that mice with a disease resembling a mild form of spinal muscular atrophy (SMA) known as SMA type 3 showed more production of a needed protein in their spinal cords and more normal-looking ears and tails after treatment with a gene-modifying molecule that researchers hope could become a treatment for human SMA.

About the new findings

Pages