Age-related changes in peroxisomal membrane protein 70 and superoxide dismutase 1 in transgenic G93A mice.
Peroxisomal membrane protein 70 (PMP70) and Cu/Zn superoxide dismutase (SOD1) were examined in the spinal cords of transgenic (Tg) mice expressing a human mutant SOD1 protein (G93A) and their age-matched controls at 8, 20 and 32 weeks by immunohistochemistry. At pre-symptomatic 20 weeks and symptomatic 32 weeks, PMP70 was reduced in the cytoplasm of motor neurons in Tg animals and increased in glial cells in anterior horn at late age. SOD1 showed a progressive increase of dot-like deposits in the neuropil of anterior horn of Tg mice, and a late decrease of signal intensity in the white matter and motor neurons at 32 weeks. It is conceivable that reduction of PMP70 might underlie decrease in peroxisomal functions and increase in oxidative stress that is well documented in this animal model.
Ilieva
Hristelina S
HS
Department of Neurology, Graduate School of Medicine and Dentistry, Okayama University, 2-5-1 Shikata-cho, Okayama 700-8558, Japan.
Nagano
Isao
I
Murakami
Tetsuro
T
Shiote
Mito
M
Manabe
Yasuhiro
Y
Abe
Koji
K
D018528:ATP-Binding Cassette Transporters; D000367:Age Factors; D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism*; D000818:Animals; D000870:Anterior Horn Cells / Q000378:metabolism*; D004195:Disease Models, Animal; D005227:Fatty Acids / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D007395:Interneurons / Q000378:metabolism; D008565:Membrane Proteins / Q000378:metabolism*; D051379:Mice; D008822:Mice, Transgenic; D020675:Peroxisomes / Q000378:metabolism*; D013376:Substantia Gelatinosa / Q000166:cytology; D013482:Superoxide Dismutase / Q000235:genetics*; D000072105:Superoxide Dismutase-1
C063305:ABCD3 protein, human; D018528:ATP-Binding Cassette Transporters; C489271:Abcd3 protein, mouse; D005227:Fatty Acids; D008565:Membrane Proteins; C000606290:SOD1 protein, human; C000606291:Sod1 protein, mouse; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1