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Publication

Glycoprotein nonmetastatic melanoma protein B ameliorates skeletal muscle lesions in a SOD1G93A mouse model of amyotrophic lateral sclerosis.

Journal of neuroscience research | 2015

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive loss of motor neurons and subsequent muscular atrophy. The quality of life of patients with ALS is significantly improved by ameliorating muscular symptoms. We previously reported that glycoprotein nonmetastatic melanoma protein B (GPNMB; osteoactivin) might serve as a target for ALS therapy. In the present study, superoxide dismutase 1/glycine residue 93 changed to alanine (SOD1(G93A) ) transgenic mice were used as a model of ALS. Expression of the C-terminal fragment of GPNMB was increased in the skeletal muscles of SOD1(G93A) mice and patients with sporadic ALS. SOD1(G93A) /GPNMB transgenic mice were generated to determine whether GPNMB expression ameliorates muscular symptoms. The weight and cross-sectional area of the gastrocnemius muscle, number and cross-sectional area of myofibers, and denervation of neuromuscular junctions were ameliorated in SOD1(G93A) /GPNMB vs. SOD1(G93A) mice. Furthermore, direct injection of a GPNMB expression plasmid into the gastrocnemius muscle of SOD1(G93A) mice increased the numbers of myofibers and prevented myofiber atrophy. These findings suggest that GPNMB directly affects skeletal muscle and prevents muscular pathology in SOD1(G93A) mice and may therefore serve as a target for therapy of ALS.

Pubmed ID: 26140698 RIS Download

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This is a list of tools and resources that we have found mentioned in this publication.

Goat anti-Rabbit IgG (H+L) Secondary Antibody, HRP (antibody)

RRID:AB_1185567

This polyclonal secondary targets IgG (H+L)

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alpha bungarotoxin (antibody)

RRID:AB_2313931

This unknown targets

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Goat Anti-Mouse Osteoactivin / gpnmb Polyclonal antibody, Unconjugated (antibody)

RRID:AB_2112934

This polyclonal targets Mouse Osteoactivin / GPNMB

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SYP (D-4) (antibody)

RRID:AB_628311

This monoclonal targets SYP

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Rabbit Anti-GAPDH Monoclonal Antibody, Unconjugated, Clone 14C10 (antibody)

RRID:AB_561053

This recombinant monoclonal targets GAPDH

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Goat Anti-Human Osteoactivin / gpnmb Polyclonal antibody, Unconjugated (antibody)

RRID:AB_416615

This polyclonal targets Human Osteoactivin / GPNMB

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Rabbit Anti-Mouse IgG, F(ab')2 Fragment Antibody, Alexa Fluor?? 488 Conjugated (antibody)

RRID:AB_1500750

This unknown targets Mouse IgG, F(ab& 39;)2 Fragment

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Rabbit anti-Goat IgG (H+L) Secondary Antibody, HRP (antibody)

RRID:AB_228395

This polyclonal secondary targets IgG (H+L)

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Anti-HaloTag pAb (antibody)

RRID:AB_713650

This polyclonal targets HaloTag pAb

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ImageJ (software resource)

RRID:SCR_003070

Open source Java based image processing software program designed for scientific multidimensional images. ImageJ has been transformed to ImageJ2 application to improve data engine to be sufficient to analyze modern datasets.

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