St. Jude scientists say FKHR protein causes primitive cells called myoblasts to fuse, while deficiency of FKHR contributes to muscle cancer
Investigators at St. Jude Childrens Research Hospital have discovered that a protein causing mature cells to commit suicide also helps primitive muscle cells called myoblasts fuse together, allowing them to develop into muscles. The finding of this unexpected new role for the protein, called FKHR, suggests that future research might offer clues to how mutated forms of this molecule cause a form of muscle cancer in children called rhabdomyosarcoma.
Rhabdomyosarcoma is a highly malignant tumor arising when primitive cells called myoblasts do not fuse and differentiate into muscle, but rather grow uncontrollably. Rhabdomyosarcoma accounts for 5-8 percent of childhood cancers and is usually diagnosed within the first 10 years of life. The most aggressive form of rhabdomyosarcoma is the alveolar type, which usually affects muscles in the extremities or trunk. The other most common type, embryonal rhabdomyosarcoma, occurs in the head and neck region and genitourinary tract. The discovery of the role of FKHR is important because of the protein’s link to a childhood cancer. Mutations of the FKHR gene occur when a piece of either of two genes—PAX3 or PAX7—break away from their own chromosomes and attach to FKHR forming PAX3-FKHR or PAX7-FKHR “fusion genes.” These genes then cause rhabdomyosarcoma. Understanding the normal role of FKHR in myoblasts could help explain how the mutated FKHR genes cause cancer, according to the St. Jude researchers.
Bonnie Cameron | EurekAlert!
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