A transgenic mouse model for human neurofibromatosis

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

Research output: Contribution to journalArticle

179 Citations (Scopus)

Abstract

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
JournalScience
Volume237
Issue number4820
DOIs
StatePublished - Jan 1 1987

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Neurofibromatoses
Transgenic Mice
Human T-lymphotropic virus 1
Neoplasms
Neurofibromatosis 1
tat Genes
Retroviridae Infections
Tropical Spastic Paraparesis
tat Gene Products
Nerve Sheath Neoplasms
Terminal Repeat Sequences
Virus Diseases
Nervous System Diseases
Peripheral Nerves
Nude Mice
Nervous System
Multiple Sclerosis
Cultured Cells
Fibroblasts
Phenotype

ASJC Scopus subject areas

  • General

Cite this

Hinrichs, S. H., Nerenberg, M., Reynolds, R. K., Khoury, G., & Jay, G. (1987). A transgenic mouse model for human neurofibromatosis. Science, 237(4820), 1340-1343. https://doi.org/10.1126/science.2888191

A transgenic mouse model for human neurofibromatosis. / Hinrichs, Steven Heye; Nerenberg, Michael; Reynolds, R. Kay; Khoury, George; Jay, Gilbert.

In: Science, Vol. 237, No. 4820, 01.01.1987, p. 1340-1343.

Research output: Contribution to journalArticle

Hinrichs, SH, Nerenberg, M, Reynolds, RK, Khoury, G & Jay, G 1987, 'A transgenic mouse model for human neurofibromatosis', Science, vol. 237, no. 4820, pp. 1340-1343. https://doi.org/10.1126/science.2888191
Hinrichs SH, Nerenberg M, Reynolds RK, Khoury G, Jay G. A transgenic mouse model for human neurofibromatosis. Science. 1987 Jan 1;237(4820):1340-1343. https://doi.org/10.1126/science.2888191
Hinrichs, Steven Heye ; Nerenberg, Michael ; Reynolds, R. Kay ; Khoury, George ; Jay, Gilbert. / A transgenic mouse model for human neurofibromatosis. In: Science. 1987 ; Vol. 237, No. 4820. pp. 1340-1343.
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