Protein Information

ID 326
Name NF2
Synonyms ACN; BANF; Merlin; Moesin ezrin radixin like protein; NF2; Neurofibromatosis type 2; Neurofibromatosis 2; Neurofibromin 2…

Compound Information

ID 332
Name 4-aminopyridine
CAS 4-pyridinamine

Reference

PubMed Abstract RScore(About this table)
10964617 Rosenbaum C, Kamleiter M, Grafe P, Kluwe L, Mautner V, Muller HW, Hanemann CO: Enhanced proliferation and potassium conductance of Schwann cells isolated from NF2 schwannomas can be reduced by quinidine. Neurobiol Dis. 2000 Aug;7(4):483-91.
Neurofibromatosis type 2 (NF2) is an autosomal dominant disease that is characterized mainly by schwannomas, as well as menigiomas and gliomas. The NF2 gene product merlin/schwannomin acts as a tumor suppressor. Schwann cells derived from NF2 schwannomas showed an enhanced proliferation rate, and electrophysological studies revealed larger K (+) outward currents as compared with controls. Schwann cells isolated from schwannomas of NF2 patients or multiorgan donors were treated with different concentrations of the K (+) current blockers quinidine, tetraethylammonium chloride, and 4-aminopyridine and K (+) outward currents and proliferation rates of these cells were compared. K (+) outward currents of both cell types can be blocked by quinidine. Importantly, treatment with quinidine reduces proliferation of NF2 Schwann cells in a concentration dependent manner but did not reduce proliferation of normal Schwann cells. Therefore, the use of quinidine or quinidine-like components would possibly provide a novel adjuvant therapeutic option for NF2 patients to slow down or freeze growth of schwannomas.
11(0,0,1,6)