AI Article Synopsis
- The NF2 protein, also known as merlin or schwannomin, acts as a tumor suppressor, with mutations in the NF2 gene frequently found in schwannomas and meningiomas.
- Despite the loss of merlin being crucial for tumor development, some tumors show no NF2 mutations, indicating alternate ways merlin can be inactivated.
- Recent research reveals that calpain, a protease, can cleave and degrade merlin, highlighting the role of the calpain system in the tumorigenesis of certain schwannomas and meningiomas.
Article Abstract
The neurofibromatosis type 2 (NF2) protein, known as merlin or schwannomin, is a tumor suppressor, and the NF2 gene has been found to be mutated in the majority of schwannomas and meningiomas, including both sporadically occurring and familial NF2 cases. Although the development of these tumors depends on the loss of merlin, the presence of tumors lacking detectable NF2 mutations suggests different mechanisms for inactivating merlin. Recent studies have demonstrated cleavage of merlin by calpain, a calcium-dependent neutral cysteine protease, and marked activation of the calpain system resulting in the degradation of merlin in these tumors. Increased turnover of merlin by calpain in some schwannomas and meningiomas exemplifies tumorigenesis linked to the calpain-mediated proteolytic pathway.
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| http://dx.doi.org/10.1046/j.1440-1789.2000.00326.x | DOI Listing |
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