Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease
Article Abstract:
Transgenic mice are used to prove the theory that forcing neurons to increase expression of neurofilaments can lead to the morphologic and pathologic properties of human motor neuron disease, such as excess of neurofilaments in perikarya and proximal axons, and increased axonal degeneration. The study indicates the role of primary cytoskeleton changes in causing diseases like amyotrophic lateral sclerosis, and implies neurofilament overaccumulation as a factor in the suggested pathogenetic sequence that leads to neutron degeneration. The susceptibility of neurons to other injuries may also increase.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1993
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An essential cytoskeletal linker protein connecting actin microfilaments to intermediate filaments
Article Abstract:
An analysis of the bullous pemphigoid antigen 1 n (BPAG1n) protein showed that it possessed functional actin- and neurofilament (NF)-binding sites at opposite ends of its coiled-coil rod. BPAG1n, which accumulates along the length of sensory axons, represents a novel class of cytoskeletal protein that plays a significant role of linking actin and intermediate filament arrays in vivo. Results show the possibility that recessive BPAG1 mutations are related to severe human neurodegenerative disorders.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1996
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Neuronal growth and death: order and disorder in the axoplasm
Article Abstract:
Neurons grow by elongation and radial growth. Their size is dictated by the accumulation of actin, myosin and microtubules along their length and neurofilaments across their width. Growth is limited by the rate at which growth components are transported along the axon by ATP-dependent microtubule motors. Abnormal neurofilament accumulation has been implicated in motor neuron diseases such as amyotrophic lateral sclerosis.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1996
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