Scientists explore bone marrow disease

BERKELEY, Calif., Oct. 12 (UPI) -- U.S. researchers say they've discovered a molecular mechanism underlying bone marrow failure in the X-linked human disease dyskeratosis congenital, or DC.

DC is a rate, progressive bond marrow failure syndrome that is fatal early in life.

Drs. Judy Wong and Kathleen Collins at the University of California-Berkeley, working with cells derived from DC patients, have demonstrated X-linked DC cells are unable to renew themselves because they cannot maintain sufficiently long telomeres to support proliferation.

The researchers say they found those defects in telomere maintenance are caused by a reduction in telomerase RNA, rather than a deficiency in ribosome biogenesis, as some other groups have hypothesized.

Collins said she is hopeful "our studies to understand disease mechanism will aid ongoing clinical efforts to develop disease therapies."

The research appears in the Oct. 15 issue of the journal Genes & Development.