Nerve growth factor receptor immunoreactivity on the tunica adventitia of intramuscular blood vessels in childhood muscular dystrophies.
Muscle tissues from cases of childhood neuromuscular disorders were examined immunohistochemically and immunoelectrophoretically using a monoclonal antibody against the human nerve growth factor receptor (NGFR). Strong NGFR immunoreactivity on the tunica adventitia of blood vessels and proliferating peripheral nerve endings in biopsied muscle specimens from muscular dystrophy patients was observed, but it was almost completely absent in specimens from non-diagnostic controls and cases of other neuromuscular disorders. This suggests a process in the sympathetic nervous system involving blood vessels in muscular dystrophies. Immunoblot analysis failed to show a band corresponding to 70-75 kd, the reported molecular size of the NGFR, but showed a clear band corresponding to 25 kd in muscular dystrophy patients, which is assumed to be a detached amino-terminal domain of the NGFR.
Zhao
J
J
Department of Child Development, Kumamoto University Medical School, Japan.
Yoshioka
K
K
Miike
T
T
Kageshita
T
T
Arao
T
T
D000293:Adolescent; D000911:Antibodies, Monoclonal; D001808:Blood Vessels / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008297:Male; D008970:Molecular Weight; D009132:Muscles / Q000473:pathology*; D009136:Muscular Dystrophies / Q000276:immunology / Q000473:pathology; D009468:Neuromuscular Diseases / Q000276:immunology / Q000473:pathology*; D010525:Peripheral Nerves / Q000473:pathology; D011956:Receptors, Cell Surface / Q000276:immunology / Q000378:metabolism*; D017475:Receptors, Nerve Growth Factor
D000911:Antibodies, Monoclonal; D011956:Receptors, Cell Surface; D017475:Receptors, Nerve Growth Factor