The role of nerve growth factor (NGF) and its receptors in the physiology of skeletal muscles has not been exten- sively studied in animal models. We describe the produc- tion of transgenic lines of mice expressing a neutralizing antibody against NGF (aD11) and the morphological and histochemical analysis of skeletal muscles from adult and aged anti-NGF mice. This study reveals that the chronic deprivation of NGF results in a decreased size of myoﬁbers of dorsal and hindlimb muscles in adult but not in postnatal day (P)2 mice. In myoﬁbers from adult anti-NGF mice, the presence of central nuclei, vacuolization of the cyto- plasm, and inﬂammatory cell inﬁltration was observed. The immunohistochemical analysis of these muscular ﬁbers re- vealed an upregulation of p75 expression, a decrease in adenosine triphosphatase (ATP)ase activity, and a subsar- colemmal Congo Red-positive staining. Immunostaining with an antibody against amyloid precursor protein showed an increased labeling of the cytoplasm of myoﬁbers from adult and aged anti-NGF mice. These features are reminis- cent of human myopathies, such as inclusion body myosi- tis. We conclude that NGF deﬁcits might be relevant for a class of human myopathies.
|Titolo:||Muscular dystrophy in adult and aged anti-NGF transgenic mice resembles an inclusion body myopathy|
|Data di pubblicazione:||2000|
|Appare nelle tipologie:||1.1 Articolo in rivista|