Format

Send to

Choose Destination
Neuropathol Appl Neurobiol. 2015 Feb;41(2):227-44. doi: 10.1111/nan.12123.

Galectin-1 deficiency improves axonal swelling of motor neurones in SOD1(G93A) transgenic mice.

Author information

1
Division of Neurofunctional Genomics, Department of Immunobiology and Neuroscience, Medical Institute of Bioregulation, Kyushu University, Fukuoka, Japan; Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

Abstract

AIMS:

Galectin-1, a member of the β-galactoside-binding lectin family, accumulates in neurofilamentous lesions in the spinal cords of both sporadic and familial amyotrophic lateral sclerosis (ALS) patients with a superoxide dismutase 1 gene (SOD1) mutation (A4V). The aim of this study was to evaluate the roles of endogenous galectin-1 in the pathogenesis of ALS.

METHODS:

Expression of galectin-1 in the spinal cord of mutant SOD1 transgenic (SOD1(G93A) ) mice was examined by pathological analysis, real-time RT-PCR and Western blotting. The effects of galectin-1 deficiency were evaluated by cross-breeding SOD1(G93A) mice with galectin-1 null (Lgals1(-/-) ) mice.

RESULTS:

Before ALS-like symptoms developed in SOD1(G93A) /Lgals1(+/+) mice, strong galectin-1 immunoreactivity was observed in swollen motor axons and colocalized with aggregated neurofilaments. Electron microscopic observations revealed that the diameters of swollen motor axons in the spinal cord were significantly smaller in SOD1(G93A) /Lgals1(-/-) mice, and there was less accumulation of vacuoles compared with SOD1(G93A) /Lgals1(+/+) mice. In symptomatic SOD1(G93A) /Lgals1(+/+) mice, astrocytes surrounding motor axons expressed a high level of galectin-1.

CONCLUSIONS:

Galectin-1 accumulates in neurofilamentous lesions in SOD1(G93A) mice, as previously reported in humans with ALS. Galectin-1 accumulation in motor axons occurs before the development of ALS-like symptoms and is associated with early processes of axonal degeneration in SOD1(G93A) mice. In contrast, galectin-1 expressed in astrocytes may be involved in axonal degeneration during symptom presentation.

KEYWORDS:

SOD1; amyotrophic lateral sclerosis; axonal degeneration; axonal spheroid; galectin-1

PMID:
24707896
DOI:
10.1111/nan.12123
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Wiley
Loading ...
Support Center