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Effects of rare, devastating disease linked to shrinking of cells’ telomeres

Scientists have found that much of the widespread damage that the rare genetic disease ataxia telangiectasia, or AT, wreaks on the body results from the progressive shortening of telomeres, the structures that cap the ends of a cell's chromosomes. In genetically altered mice, the researchers found that the shortening of telomeres led to a "crisis" that disrupted chromosomes "like a hand grenade thrown into the cell," as one scientist put it. The resulting cellular chaos was manifested throughout the rodents' bodies by the loss of reparative stem cells that different organs normally have in reserve, producing symptoms of premature aging such as hair loss and slow wound healing, and early death.

Gene signature identifies leukemia patients who should avoid transplants

An international team of researchers has used a gene test to identify certain patients with adult T-cell acute lymphoblastic leukemia (ALL) who can be successfully treated with chemotherapy alone and should not be subjected to the rigors of bone marrow transplants. The researchers found that these patients survived for at least three years after being treated with intensive chemotherapy. It was previously known that only slightly over half of the patients with this disease could be cured with chemotherapy. Adult ALL patients often undergo transplants in an effort to beat back the stubborn disease. Until now there was no way to identify those who have a more favorable outlook and shouldn't undergo risky bone marrow transplantation.

Thalidomide-like drug appears to help bone cancer patients

A drug similar to thalidomide has been found to be promising with fewer side effects for treating patients with recurrent multiple myeloma, an incurable form of bone marrow cancer, according to early data from a clinical study. The drug, an analog of thalidomide, was developed to be more potent than thalidomide, while reducing some of thalidomide's dose limiting side effects. Laboratory studies have shown that CC-5013 not only kills myeloma cells by triggering their innate self-destruct mechanism but also inhibits the myeloma cells ability to localize and grow in the bone marrow. Moreover, it appears to have anti-angiogenic effects and stimulates the immune system to attack myeloma.

Researchers Identify Cause of Aggressive Childhood Cancer

Researchers have generated a mouse model of a new type of tumor suppressor gene that triggers a rapidly advancing cancer that affects children. The discovery of the fast-onset cancers that result from inactivation of the gene and the technique used to generate the model will likely prove useful in studying genes involved in other forms of cancer.

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