Tumorigenesis in neurofibromatosis: new insights and potential therapies

Reed, Nevada W.; Gutmann, David H.

doi:10.1016/s1471-4914(01)01955-4

Cited by 69 publications

(35 citation statements)

References 43 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…NF2 inactivation and loss of merlin expression are associated with the development of schwannoma and meningioma tumors (reviewed by Reed and Gutmann, 2001). Moreover, the NF2 gene has also been implicated in the development of non-NF2-associated Merlin interacts with several molecules, including NHE-RF, βII-spectrin, CD44, other ERM proteins, SCHIP-1, HRS, actin and syntenin, which may affect merlin function as a growth suppressor.…”

Section: Merlin In Tumorigenesis and Developmentmentioning

confidence: 99%

Protein 4.1 tumor suppressors: getting a FERM grip on growth regulation

Sun

Robb

Gutmann

2002

Journal of Cell Science

Self Cite

166

140

View full text Add to dashboard Cite

Members of the Protein 4.1 superfamily have highly conserved FERM domains that link cell surface glycoproteins to the actin cytoskeleton. Within this large and constantly expanding superfamily, at least five subgroups have been proposed. Two of these subgroups, the ERM and prototypic Protein 4.1 molecules, include proteins that function as tumor suppressors. The ERM subgroup member merlin/schwannomin is inactivated in the tumor-predisposition syndrome neurofibromatosis 2 (NF2), and the prototypic 4.1 subgroup member,Protein 4.1B, has been implicated in the molecular pathogenesis of breast,lung and brain cancers. This review focuses on what is known of mechanisms of action and critical protein interactions that may mediate the unique growth inhibitory signals of these two Protein 4.1 tumor suppressors. On the basis of insights derived from studying the NF2 tumor suppressor, we propose a model for merlin growth regulation in which CD44 links growth signals from plasma membrane to the nucleus by interacting with ERM proteins and merlin.

show abstract

Section: Merlin In Tumorigenesis and Developmentmentioning

confidence: 99%

Protein 4.1 tumor suppressors: getting a FERM grip on growth regulation

Sun

Robb

Gutmann

2002

Journal of Cell Science

Self Cite

166

140

View full text Add to dashboard Cite

show abstract

“…Neurofibromatosis type 1 (NF1) and type 2 (NF2), which affect one in 3500 and one in 30 000À42 000 individuals, respectively, are autosomal-dominant inherited diseases, and their pathological origins are neural and neural crest-derived cells (reviewed in Enzinger and Weiss, 1988;Evans et al, 2000;Reed and Gutmann, 2001). Patients have a propensity for developing both benign and malignant tumors, and characteristic pigment abnormality.…”

Section: Introduction Results and Discussionmentioning

confidence: 99%

Conditional N-rasG12V expression promotes manifestations of neurofibromatosis in a mouse model

et al. 2007

View full text Add to dashboard Cite

Human clinical neurofibromatosis type 1 (NF1) and type 2 (NF2) result from mutations and inactivation of neurofibromin and merlin genes, respectively, which negatively regulate Ras pathways. To evaluate the contribution of N-Ras activity to the development of NF, we generated a novel transgenic mouse expressing oncogenic N-ras specifically in central nerve cells, neural crest-derived cells and lens epithelial cells. Soon after birth, the mouse skin showed hyperpigmentation of the epidermis and melanin-laden macrophages in the dermis, as observed in the cafe´-au-lait spots of human cases. At 3 months of age, all the mice had neurofibromas in the skin and neurofibroma-like tumors with structure similar to WagnerMeissner bodies in the adrenal medulla. At 4 months of age, all the mice developed subcapsular cataract. In the 5th month, some developed protruding dermal neurofibromas involving subcutaneous fat. However, plexiform neurofibroma, schwannoma, astrocytoma and pheochromocytoma were not observed in the mice, suggesting a requirement for signal(s) other than the activated N-Ras pathway to induce these tumors. Thus, the activated N-Ras signal may be a main pathway for the development of the disease phenotypes characteristic of NF.

show abstract

“…These syndromes are associated with benign tumors of the nervous system, pigmentary lesions, malignant tumors of the peripheral nerve sheath (MPNST), gliomas and myelodysplastic syndromes of the blood (Reed and Gutmann, 2001). NF2 mutations have been found in sporadic schwannomas, meningiomas, melanomas and mesotheliomas (Reed and Gutmann, 2001;Xiao et al, 2003).…”

Section: Neurofibromin and Merlinmentioning

confidence: 99%

Tumor suppressor interactions with microtubules: keeping cell polarity and cell division on track

Hernández

Tirnauer

2010

Disease Models &Amp; Mechanisms

View full text Add to dashboard Cite

Tumor suppressor proteins protect cells and tissues from malignant transformation. Among their diverse actions, many of these proteins interact with the microtubule cytoskeleton. This review focuses on the interactions of several tumor suppressors with microtubules and speculates on how disruption of microtubule-dependent processes may contribute to cancer development and spread. We conclude that several tumor suppressors stabilize microtubules and organize microtubule arrays, functions that are likely to be important in preventing tumorigenesis. How tumor suppressors link microtubule stability with cell fate, and how their mutation affects the response of cancer cells to anti-microtubule chemotherapy drugs, remains unclear; these should prove fertile areas for future research.

show abstract

Tumorigenesis in neurofibromatosis: new insights and potential therapies

Cited by 69 publications

References 43 publications

Protein 4.1 tumor suppressors: getting a FERM grip on growth regulation

Protein 4.1 tumor suppressors: getting a FERM grip on growth regulation

Conditional N-rasG12V expression promotes manifestations of neurofibromatosis in a mouse model

Tumor suppressor interactions with microtubules: keeping cell polarity and cell division on track

Contact Info

Product

Resources

About