Mechanisms for neuronal degeneration in amyotrophic lateral sclerosis and in models of motor neuron death (Review).

Martin, Lee J.; Price, Angie; Kaiser, Adeel; Shaikh, Arif; Liu, Z

doi:10.3892/ijmm.5.1.3

Cited by 62 publications

(62 citation statements)

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“…Dysregulated apoptotic cell death is held accountable for several diseases of the nervous system, and particularly within the group of neurodegenerative diseases, apoptosis has been implicated as a potentially crucial pathogenetic mechanism. 1,[15][16][17][18] Despite the considerable progress in the knowledge about pathological features of neurological diseases, the therapeutic options are currently limited. Therefore, a need exists for additional insight into the mechanisms of how life and death decisions are regulated in adult neurons.…”

Section: Discussionmentioning

confidence: 99%

Bifunctional apoptosis inhibitor (BAR) protects neurons from diverse cell death pathways

et al. 2003

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The bifunctional apoptosis regulator (BAR) is a multidomain protein that was originally identified as an inhibitor of Baxinduced apoptosis. Immunoblot analysis of normal human tissues demonstrated high BAR expression in the brain, compared to low or absent expression in other organs. Immunohistochemical staining of human adult tissues revealed that the BAR protein is predominantly expressed by neurons in the central nervous system. Immunofluorescence microscopy indicated that BAR localizes mainly to the endoplasmic reticulum (ER) of cells. Overexpression of BAR in CSM 14.1 neuronal cells resulted in significant protection from a broad range of cell death stimuli, including agents that activate apoptotic pathways involving mitochondria, TNFfamily death receptors, and ER stress. Downregulation of BAR by antisense oligonucleotides sensitized neuronal cells to induction of apoptosis. Moreover, the search for novel interaction partners of BAR identified several candidate proteins that might contribute to the regulation of neuronal apoptosis (HIP1, Hippi, and Bap31). Taken together, the expression pattern and functional data suggest that the BAR protein is involved in the regulation of neuronal survival.

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Section: Discussionmentioning

confidence: 99%

Bifunctional apoptosis inhibitor (BAR) protects neurons from diverse cell death pathways

et al. 2003

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“…Because not all ALS patients have defective genes, the results of such attempts are often verified by studying motor neuron death (common to all cases of ALS) in an animal model of acute peripheral nerve axotomy (8,9). The only drug currently used to slow down the progression of ALS, although with only modest effect, is riluzole, a putative blocker of glutamate release (10,11).…”

mentioning

confidence: 99%

Therapeutic vaccine for acute and chronic motor neuron diseases: Implications for amyotrophic lateral sclerosis

Angelov

Waibel

Guntinas‐Lichius

et al. 2003

Proc. Natl. Acad. Sci. U.S.A.

179

104

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Therapeutic vaccination with Copaxone (glatiramer acetate, Cop-1) protects motor neurons against acute and chronic degenerative conditions. In acute degeneration after facial nerve axotomy, the number of surviving motor neurons was almost two times higher in Cop-1-vaccinated mice than in nonvaccinated mice, or in mice injected with PBS emulsified in complete Freund's adjuvant (P < 0.05). In mice that express the mutant human gene Cu͞Zn superoxide dismutase G93A (SOD1), and therefore simulate the chronic human motor neuron disease amyotrophic lateral sclerosis, Cop-1 vaccination prolonged life span compared to untreated matched controls, from 211 ؎ 7 days (n ‫؍‬ 15) to 263 ؎ 8 days (n ‫؍‬ 14; P < 0.0001). Our studies show that vaccination significantly improved motor activity. In line with the experimentally based concept of protective autoimmunity, these findings suggest that Cop-1 vaccination boosts the local immune response needed to combat destructive self-compounds associated with motor neuron death. Its differential action in CNS autoimmune diseases and neurodegenerative disorders, depending on the regimen used, allows its use as a therapy for either condition. Daily administration of Cop-1 is an approved treatment for multiple sclerosis. The protocol for non-autoimmune neurodegenerative diseases such as amyotrophic lateral sclerosis, remains to be established by future studies.

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“…28 Superoxide dismutase is an enzyme that scavenges oxygen free radicals and protects cells from oxidative damage. Initially, mutations in SOD were predicted to alter the enzymatic properties of this protein.…”

Section: Amyotropic Lateral Sclerosis (Als)mentioning

confidence: 99%

Neurodegeneration: diseases of the cytoskeleton?

McMurray

2000

Cell Death Differ

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Mechanisms for neuronal degeneration in amyotrophic lateral sclerosis and in models of motor neuron death (Review).

Cited by 62 publications

References 0 publications

Bifunctional apoptosis inhibitor (BAR) protects neurons from diverse cell death pathways

Bifunctional apoptosis inhibitor (BAR) protects neurons from diverse cell death pathways

Therapeutic vaccine for acute and chronic motor neuron diseases: Implications for amyotrophic lateral sclerosis

Neurodegeneration: diseases of the cytoskeleton?

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