Neurofilament 70 kD

GWB-BE1FFD

mouse IgG1

Each vial contains 100& #956; l 1 mg/ml purified monoclonal antibody in PBS containing 0. 09% sodium azide. Like most other intermediate filament proteins (IFPs). the expression of the different neuronal IFPs is both tissue-specific and developmentally regulated. The neurofilament (NF) triplet proteins (70. 160. and 200 kDa) occur in both the central and peripheral nervous system and are normally restricted to neurons. The 70 kDa NF-protein can self-assemble into a filamentous structure. whereas the 160 kDa and 200 kDa NF-proteins require the presence of the 70 kDa NF-protein to co-assemble. All three NF proteins can be detected by immunohistochemical methods at day 9 or 10 after gestation in the mouse embryo. Although IFPs of the neurofilament type are normally restricted to neurons. there are reports on their expression in non-neuronal cells as well. For example. in heart conduction myocytes NF proteins are expressed together with desmin. In tumorpathology ganglioneuroblastomas and some of the other neuroblastomas are strongly positive with the neurofilament antisera. Also. some neuro-endocrine malignancies may show NF positivity. In cell cultures of neural tissues the neurofilament antibodies can monitor in vitro differentiation.

Interacts with RGNEF (By similarity).

O-glycosylated (By similarity).

Defects in NEFL are the cause of Charcot-Marie-Tooth disease type 2E (CMT2E) [MIM:607684]. CMT2E is an autosomal dominant form of Charcot-Marie-Tooth disease type 2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations normal or slightly reduced nerve conduction velocities and progressive distal muscle weakness and atrophy.

Belongs to the intermediate filament family. 1. Kluck. P. van Muijen. G. N. van der Kamp. A. W. Tibboel. D. van Hoorn. W. A. Warnaar. S. O. and Molenaar. J. C. (1984). Hirschsprung\' s disease studied with monoclonal antineurofilament antibodies on tissue sections. Lancet 1. 652-4. 2. van Muijen. G. N. Ruiter. D. J. van Leeuwen. C. Prins. F. A. Rietsema. K. and Warnaar. S. O. (1984). Cytokeratin and neurofilament in lung carcinomas. Am J Pathol 116. 363-9. 3. Breckenridge. L. J. Sommer. I. U. and Blackshaw. S. E. (1997). Developmentally regulated markers in the postnatal cervical spinal cord of the opossum Monodelphis domestica. Brain Res Dev Brain Res 103. 47- 57.