The research, published in todays edition of Science, reveals that tumors in neurofibromatosis 1 (NF-1) patients form out of a particular type of cell, the Schwann cell. Neurofibromatosis, also known as von Recklinghausen disease, is a genetic disorder that causes benign tumors to form on the nerves, skin and internal organs.
Neurofibromatosis is the most common neurological disorder caused by a single gene, and there are two distinct forms: NF-1, which affects multiple organ systems, including the central and peripheral nervous systems, and is present in about 1 in 3,500 people worldwide; and NF-2, which affects only the vestibular nerve, a cranial nerve related to hearing and balance, and the membranes around the brain and spinal cord. NF-2 is present in about 1 in 40,000 people worldwide.
The benign tumors, called neurofibromas, develop into malignant tumors, called neurofibrosarcomas, in 10 percent to 15 percent of NF-1 patients. The malignant tumors do not respond well to currently available treatments.
The new research describes genetically permissive and restrictive environments for NF-1 tumor development. The tumors grow in a permissive environment, but they are nonexistent when the environment is restrictive, researchers say.
Dr. Yuan Zhu, instructor in the Center for Developmental Biology and lead author of the study, said the findings could lead to a new approach to combating NF-1. Zhu has been studying neurofibromatosis since 1995, when as a graduate student he joined the lab of Dr. Luis Parada, director of the Center for Developmental Biology and senior author of the paper.
The focus has always been on attacking the tumor cells, Zhu said. But in our findings, killing the tumor cells is not t
Contact: Wayne Carter
UT Southwestern Medical Center