Of mice, men and motor neurons

Trends in Molecular Medicine

Volumn 8, Issue 2, 2002, Pages 88-92

  Newbery, Helen J ^a   Abbott, Catherine M ^a  

^a UNIVERSITY OF EDINBURGH   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

SUPEROXIDE DISMUTASE; VASCULOTROPIN;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL EXPERIMENT; ANIMAL MODEL; CYTOSKELETON; GENE EXPRESSION; GENE MUTATION; HUMAN; MOTONEURON; MOTOR NEURON DISEASE; MOUSE; NEUROFILAMENT; NEUROMUSCULAR DISEASE; NONHUMAN; PATHOGENESIS; REVIEW; TRANSGENIC MOUSE;

ANIMALIA; MUS MUSCULUS;

EID: 0036151526     PISSN: 14714914     EISSN: None     Source Type: Journal    
DOI: 10.1016/S1471-4914(02)02283-9     Document Type: Review

References (35)

1. Genetics of amyotrophic lateral sclerosis
2. Mice, the motor system, and human motor neuron pathology
3. Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury
4. Transgenic mouse model for familial amyotrophic lateral sclerosis with superoxide dismutase-1 mutation
5. Neuroprotective effect of creatine in a transgenic animal model of amyotrophic lateral sclerosis
6. Neuron-specific expression of mutant superoxide dismutase 1 in transgenic mice does not lead to motor impairment
7. Accumulation of phosphorylated neurofilaments in anterior horn motoneurons of amyotrophic lateral sclerosis patients
8. Delayed maturation of regenerating myelinated axons in mice lacking neurofilaments
9. Absence of the mid-sized neurofilament subunit decreases axonal calibers, levels of light neurofilament (NF-L), and neurofilament content
10. Neurofilament-dependent radial growth of motor axons and axonal organization of neurofilaments does not require the neurofilament heavy subunit (NF-H) or its phosphorylation
11. Disruption of the NF-H gene increases axonal microtubule content and velocity of neurofilament transport: Relief of axonopathy resulting from the toxin β,β′-iminodipropionitrile
12. Requirement of heavy neurofilament subunit in the development of axons with large calibers
13. Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: A mouse model of amyotrophic lateral sclerosis
14. Neurofilament subunit NF-H modulates axonal diameter by selectively slowing neurofilament transport
15. Selective degeneration of Purkinje cells with Lewy body-like inclusions in aged NFH/LacZ transgenic mice
16. Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase
17. Deregulation of Cdk5 in a mouse model of ALS: Toxicity alleviated by perikaryal neurofilament inclusions
18. Reduction of axonal caliber does not alleviate motor neuron disease caused by mutant superoxide dismutase 1
19. Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration
20. The lethal mutation of the mouse wasted (wst) is a deletion that abolishes expression of a tissue-specific isoform of translation elongation factor-1α, encoded by the Eefla2 gene
21. Ultrastructural, morphometric, and immunocytochemical study of anterior horn cells in mice with 'wasted' mutation
22. Interaction of ZPR1 with translation elongation factor-1α, in proliferating cells
23. Spinal muscular atrophy disrupts the interaction of ZPR1 with the SMN protein
24. Elongation factor 1α, translation and the cytoskeleton
25. Identification of the mouse neuromuscular degeneration gene and mapping of a second site suppressor allele
26. Mutations in the gene encoding immunoglobulin mu-binding protein 2 cause spinal muscular atrophy with respiratory distress type 1
27. Genetic mapping of a mouse modifier gene that can prevent ALS onset
28. Overexpression of neurofilament subunit NF-L and NF-H extends survival of a mouse model for amyotraphic lateral sclerosis
29. Absence of neurofilaments reduces the selective vulnerability of motor neurons and slows disease caused by a familial amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutant
30. Extra neurofilament NF-L subunits rescue motor neuron disease caused by overexpression of the human NF-H gene in mice
31. Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease
32. Formation of intermediate filament protein aggregates with disparate effects in two transgenic mouse models lacking the neurofilament light subunit
33. Late onset death of motor neurons in mice overexpressing wild-type peripherin
34. The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis
35. A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2