IMMUNOREACTIVITY OF PLATELET-DERIVED GROWTH
FACTOR AND ITS ALPHA-RECEPTOR IN BIOPSIES MUSCLES OF PROGRESSIVE MUSCULAR
DYSTROPHIES
Zhao Yajuan1,Haginoya
K2,Linuma K2
1.Department of
Pediatrics,Second Clinical College of China Medical University
2.Department of
Pediatrics,Tohoku University School of Medicine, Sendai, Japan
Objective: Many growth factors contribute to the
regeneration of muscle fibers in progressive muscular hypothesis that
activation of the Platelet-derived growth factor (PDGF)-A and its
alpha-receptor pathway is not reported. Immunohistochemical examination of
progressive muscular dystrophy and controlling muscle biopsies were
performed to investigate the presence of PDGF-A and its alpha-receptor in
progressive muscular dystrophy muscle.
Methods: Polyclonal antibody against human
PDGF-A and polyclonal antibody against human PDGF alpha-receptor were from
Santa Cruz Biotechnology.Immunocalizayed with A.B.C. method and
immunofloresent assay. In control experiments anti-PDGF-A and its alpha-receptor
were replaced with nonimmune normal rabbit IgG.Immunoabsortion test was
done with PDGF-A peptide.For the purpese of deteceing Nuromuscular Jaction
and regenerative muscle fibers,double staning was perfomed
alpha-bungarotoxin and anti-desmin monobody IgG.
Materials: biceps muscle of arm biopsy specimens
of 31 patients suffering from progressive muscular dystrophy 6 FCMD, 3
nFCMD with positive of merosin, 7 DMD, 1 BMD, 5 mitochondria myopathy, 2
congenital minproressive myopathy, 1 neuropathy, 2 wardnig-hoffmann, 4
myositis. Confirmed by clinical examination as well as routine
histological, immunohistological and microscopic preparations were studies.
13 patients without clinical and histological evidence of a neuromuscular
disorder served as normal controls.
Results:1 The skeletal
muscle section showed positive immunoreactivity to PDGF-A in the small
fibers of FCMD ,nFCMD and DMD .Strong expression of PDGF-A in small fibers
of FCMD and nFCMD than DMD by using A.B.C. method. Lightly positive
immunoreactivity to PDGF-A was observed in the muscle sections of another
myopathy .In addition, weakly positive immunoreactivity to PDGF-A was seen
in capillary smooth muscle cells surrounding blood vessels. Areas of
intense immunoreactivity to desmin were again observed strong expression of
PDGF-A in FCMD and nFCMD by using double staining, there areas were whole
small fibers with regenerate fibers. 2.The muscle cell membranes of DMD,
FCMD,n FCMD were revealed to have PDGF-alpha receptor antigen: Parts of
perimysium and endomysium were also stained. Cytoplasm of small diameter fibers including
regenerative fibers was stained moderately. Neuromuscular junction,
vascular vessels of muscles and smaller muscles of great vessels were
stained too.
Conclusion: PDGF were strongly
discovered in progressive muscular dystrophies especially in FCMD and
nFCMD. Furthermore, PDGF alpha
receptor exist in muscular membrane and interstotia .We conclude that there
is a close relutior between PDGF and tissue changes of muscular regeneration
and fibrosis in progressive muscular dystrophy and PDGF alpha receptor may
play a physi
ological role in neuromuscular
junctio.