Abstract

This project is aimed at the understanding of the biochemistry of the neurofilament triplet polypeptides in solution. I will focus on the separation and the isolation of these polypeptides, and compare these three chains by biochemical and physical biochemical methods. The in vivo conditions for assembly and disassembly of the neurofilaments will be studied. In addition, the interactions between the neurofilament triplet and the protein subunits of the microtubules and microfilaments will be studied. Finally, the relationship between the neurofilament triplet and the axonal transport triplet will be investigated.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Research Project (R01)
Project #
5R01NS015182-07
Application #
3396021
Study Section
Neurology B Subcommittee 1 (NEUB)
Project Start
1979-07-01
Project End
1986-06-30
Budget Start
1985-07-01
Budget End
1986-06-30
Support Year
7
Fiscal Year
1985
Total Cost
Indirect Cost

  Institution

Name
New York University
Department
Type
Schools of Medicine
DUNS #
004514360
City
New York
State
NY
Country
United States
Zip Code
10012

  Publications

Liem, Ronald K H; Messing, Albee (2009) Dysfunctions of neuronal and glial intermediate filaments in disease. J Clin Invest 119:1814-24
Ching, Gee Y; Liem, Ronald K H (2009) RE1 silencing transcription factor is involved in regulating neuron-specific expression of alpha-internexin and neurofilament genes. J Neurochem 109:1610-23
Goryunov, Dmitry; Nightingale, Andrew; Bornfleth, Lorelei et al. (2008) Multiple disease-linked myotubularin mutations cause NFL assembly defects in cultured cells and disrupt myotubularin dimerization. J Neurochem 104:1536-52
Kabzinska, Dagmara; Perez-Olle, Raul; Goryunov, Dmitry et al. (2006) Is a novel I214M substitution in the NEFL gene a cause of Charcot-Marie-Tooth disease? Functional analysis using cell culture models. J Peripher Nerv Syst 11:225-31
Perez-Olle, Raul; Lopez-Toledano, Miguel A; Goryunov, Dmitry et al. (2005) Mutations in the neurofilament light gene linked to Charcot-Marie-Tooth disease cause defects in transport. J Neurochem 93:861-74
Perez-Olle, Raul; Jones, Sidonie T; Liem, Ronald K H (2004) Phenotypic analysis of neurofilament light gene mutations linked to Charcot-Marie-Tooth disease in cell culture models. Hum Mol Genet 13:2207-20
Perez-Olle, Raul; Lopez-Toledano, Miguel A; Liem, Ronald K H (2004) The G336S variant in the human neurofilament-M gene does not affect its assembly or distribution: importance of the functional analysis of neurofilament variants. J Neuropathol Exp Neurol 63:759-74
Leung, Conrad L; He, Cui Zhen; Kaufmann, Petra et al. (2004) A pathogenic peripherin gene mutation in a patient with amyotrophic lateral sclerosis. Brain Pathol 14:290-6
Leung, Conrad L; Green, Kathleen J; Liem, Ronald K H (2002) Plakins: a family of versatile cytolinker proteins. Trends Cell Biol 12:37-45
Perez-Olle, Raul; Leung, Conrad L; Liem, Ronald K H (2002) Effects of Charcot-Marie-Tooth-linked mutations of the neurofilament light subunit on intermediate filament formation. J Cell Sci 115:4937-46

Showing the most recent 10 out of 49 publications