A transgenic mouse model for human neurofibromatosis

Steven H. Hinrichs, Michael Nerenberg, R. Kay Reynolds, George Khoury, Gilbert Jay

Research output: Contribution to journalArticlepeer-review

185 Scopus citations


Human T-lymphotropic virus type 1 (HTLV-1) has been associated with the neurologic disorder tropical spastic paraparesis and possibly with multiple sclerosis. The tat gene of HTLV-1 under control of its own long terminal repeat is capable of inducing tumors in transgenic mice. The morphologic and biologic properties of these tumors indicate their close resemblance to human neurofibromatosis (von Recklinghausen's disease), the most common single gene disorder to affect the nervous system. The high spontaneous incidence of this disease, together with the diverse clinical and pathologic features associated with it, suggests that environmental factors may account for some of the observed cases. Multiple tumors developed simultaneously in the transgenic tat mice at approximately 3 months of age, and the phenotype was successfully passed through three generations. The tumors arise from the nerve sheaths of peripheral nerves and are composed of perineural cells and fibroblasts. Tumor cells from these mice adapt easily to propagation in culture and continue to express the tat protein in significant amounts. When transplanted into nude mice, these cultured cells efficiently induce tumors. Evidence of HTLV-1 infection hi patients with neural and other soft tissue tumors is needed in order to establish a link between infection by this human retrovirus and von Recklinghausen's disease and other nonlymphoid tumors.

Original languageEnglish (US)
Pages (from-to)1340-1343
Number of pages4
Issue number4820
StatePublished - 1987
Externally publishedYes

ASJC Scopus subject areas

  • General

Fingerprint Dive into the research topics of 'A transgenic mouse model for human neurofibromatosis'. Together they form a unique fingerprint.

Cite this