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Osteogenic tumours in Lkb1-deficient mice
James Robinson1, Emma Nye, Gordon Stamp
1Colorectal Cancer Genetics, Institute of Cell and Molecular Science, Barts and The London, Queen Mary's School of Medicine and Dentistry, 4 Newark Street, Whitechapel, London E1 2AT, UK.
Experimental and Molecular Pathology
|September 2, 2008
Summary
Germline LKB1 mutations cause Peutz-Jeghers Syndrome. LKB1 deficiency in mice leads to osteoblastic tumors, causing paralysis and spinal canal compression, suggesting LKB1 controls osteoblast proliferation.
Area of Science:
- Oncology
- Genetics
- Mouse Models
Background:
- Germline mutation in Liver Kinase B1 (LKB1) causes Peutz-Jeghers Syndrome (PJS) in humans, a disorder linked to cancer and gastrointestinal polyps.
- Mice with inactivating LKB1 mutations exhibit PJS-like gastrointestinal polyps and liver neoplasia.
Purpose of the Study:
- To investigate the potential role of LKB1 in bone development and tumor formation.
- To characterize the phenotype of Lkb1(+/-) mice beyond gastrointestinal and liver abnormalities.
Main Methods:
- Phenotypic analysis of Lkb1(+/-) mice on C57BL/6J and 129/sv backgrounds.
- Histopathological examination of spinal columns and long bones from affected and unaffected mice.
- Comparison with wild-type Lkb1(+/+) littermates.
Main Results:
- Approximately 2% of Lkb1(+/-) mice developed paralysis around 300 days of age.
- Serial sectioning revealed multifocal osteoblastic tumors in the spinal columns of affected mice, with infiltrative growth and osteoid formation.
- Osteoblastic proliferation and abnormal bone remodeling, including angiogenesis, were observed in the long bones of Lkb1(+/-) mice, but not in wild-type controls.
Conclusions:
- This study reports the first observation of multifocal osteoblastic tumors in Lkb1(+/-) mice.
- The findings suggest a novel role for LKB1 in controlling osteoblast proliferation, similar to PTEN.
- LKB1 deficiency may predispose to osteogenic tumor development, impacting skeletal integrity and potentially causing neurological deficits.
