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  • Open Access

HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP)-like disease and toxic epidermal necrolysis (TEN)-like disease in HTLV-1 Tax transgenic mice

  • 1Email author,
  • 1,
  • 1,
  • 1,
  • 2 and
  • 3
Retrovirology201411 (Suppl 1) :P40

https://doi.org/10.1186/1742-4690-11-S1-P40

  • Published:

Keywords

  • Transgenic Mouse
  • Toxic Epidermal Necrolysis
  • Lumbar Spinal Cord
  • Spastic Paraparesis
  • Detachment Rate

Human T-cell leukemia virus type 1 (HTLV-1) can cause an aggressive malignancy known as adult T-cell leukemia/lymphoma (ATLL), as well as inflammatory diseases such as HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). We created a transgenic mouse model of HTLV-1 infection by using the distal promoter of Lck to express tax in mature thymocytes and peripheral T lymphocytes. Major phenotypes of disease in this model were mature T cell leukemia/lymphoma similar to ATLL and an inflammatory arthropathy. While expanding the transgenic mouse colony, we also found 2.7% of transgenic mice developed a HAM/TSP-like disease and 2.4% of transgenic mice developed a toxic epidermal necrolysis (TEN)-like disease. The HAM/TSP-like disease with symmetrical paraparesis of the hind limbs in transgenic mice was not inflammatory, unlike that found in HAM/TSP patients, but instead involved the invasion of histiocytic sarcoma cells into the lumbar spinal cord from the bone marrow where they had undergone extensive proliferation. Mice with the HAM/TSP-like disease showed abnormal expression of cytokines and chemokines, including TNF and IL-6. There are no reports of spontaneous symmetrical paraparesis caused by histiocytic sarcoma in mice. TEN is characterized by a rash, bullae, and diffuse exfoliation of wide cutaneous surface areas, as seen in second-degree burns. Massive keratinocyte apoptosis is the hallmark of TEN. The TEN-like disease in transgenic mice occurred mainly in C57BL/6 background strain mice. These mice showed a skin detachment rate of greater than 30%. The pathology in transgenic mice with a TEN-like disease is currently under study and will be discussed.

Authors’ Affiliations

(1)
Division of Microbiology and Genetics, Institute of Resource Development and Analysis, Kumamoto University, Kumamoto, Japan
(2)
Department of Pathology, Japanese Red Cross Medical Center, Tokyo, Japan
(3)
Department of Safety Research on Blood and Biologics, National Institute of Infectious Diseases, Tokyo, Japan

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