Advanced glycation end product in familial amyloidotic polyneuropathy (FAP).
OBJECTIVE
Advanced glycation end products (AGE) are present in amyloid deposits in beta2-microglobulin amyloidosis, and it has been postulated that glycation of beta2-microglobulin may be involved in fibril formation. The aim of this paper was to ascertain whether AGE occur in amyloid deposits in familial amyloidotic polyneuropathy (FAP).


METHODS
Department of Medicine, Umeå University Hospital and First Department of Internal Medicine, Kumamoto University School of Medicine.


METHODS
The presence of AGE was sought immunohistochemically and biochemically in amyloid-rich tissues from patients with FAP.


METHODS
Biopsy specimens from nine patients and 10 controls were used for the immunohistochemical analysis. For amyloid preparation, vitreous samples from three FAP patients were used.


RESULTS
Immunohistochemical studies using a polyclonal anti-AGE antibody revealed positive immunoreactivity in intestinal materials, but the pattern of reactivity was unevenly distributed; it was often present in the border of amyloid deposits, or surrounding them. Non-amyloid associated immunoreactivity was also observed in a few regions of the specimens, although the AGE-positive structures were situated in areas containing amyloid deposits. Western blotting of purified amyloid from the vitreous body of FAP patients revealed a significant association of AGE with amyloid fibrils.


CONCLUSIONS
The immunoreactivity for the AGE antibody suggests that AGE may be involved in fibril formation in FAP.
Nyhlin
N
N
Gastroenterology and Hepatology Section, Department of Medicine, Umeå University Hospital, S-901 85 Umeå, Sweden.
Ando
Y
Y
Nagai
R
R
Suhr
O
O
El Sahly
M
M
Terazaki
H
H
Yamashita
T
T
Ando
M
M
Horiuchi
S
S
D000328:Adult; D000368:Aged; D017772:Amyloid Neuropathies / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D001706:Biopsy; D003238:Connective Tissue / Q000473:pathology; D005260:Female; D017127:Glycation End Products, Advanced / Q000032:analysis*; D006801:Humans; D007124:Immunoenzyme Techniques; D007413:Intestinal Mucosa / Q000473:pathology; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D009131:Muscle, Smooth, Vascular / Q000473:pathology; D014821:Vitrectomy; D014822:Vitreous Body / Q000473:pathology
D017127:Glycation End Products, Advanced