Role of serum amyloid P component for systemic amyloidosis in transgenic mice carrying human mutant transthyretin gene.
The role of serum amyloid P component (SAP) for systemic amyloidosis in a transgenic mouse model for an autosomal dominant disease, familial amyloidotic polyneuropathy (FAP), was examined. For this purpose, two lines of transgenic mouse were produced by introducing the human mutant transthyretin (TTR) gene and the human SAP gene, respectively. Two lines of transgenic mice were mated to produce double transgenic mice carrying both human mutant TTR gene and human SAP gene. The serum concentration of human SAP in these transgenic mice was about 42 micrograms/ml and was about equal to that in human control serum. In case of single transgenic mice carrying human mutant TTR gene, amyloid deposition starts at around 6 months of age, and the amount of amyloid deposition increases gradually with age. Amyloid deposition is observed in many tissues including heart, kidney and thyroid gland, where amyloid deposition is commonly observed in FAP patients. In double transgenic mice, onset, progression and tissue distribution of amyloid deposition were the same as those in single transgenic mouse. These results clearly suggest that SAP is not important for the initiation and progression of amyloid deposition.
Tashiro
F
F
Institute for Medical Genetics, Kumamoto University Medical School, Japan.
Yi
S
S
Wakasugi
S
S
Maeda
S
S
Shimada
K
K
Yamamura
K
K
D000367:Age Factors; D000686:Amyloidosis / Q000235:genetics / Q000378:metabolism*; D000818:Animals; D004195:Disease Models, Animal; D005260:Female; D015870:Gene Expression; D006801:Humans; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D009154:Mutation; D009422:Nervous System Diseases / Q000235:genetics / Q000378:metabolism; D011228:Prealbumin / Q000235:genetics*; D000683:Serum Amyloid P-Component / Q000235:genetics / Q000378:metabolism*; D014018:Tissue Distribution
D011228:Prealbumin; D000683:Serum Amyloid P-Component