In some amyloidoses, transmission by self-propagating amyloid proteins plays a critical role in the progression of the disease. Mouse senile amyloidosis is a disorder in which apolipoprotein A-II (ApoA-II) deposits as amyloid fibrils (AApoAII), and it might be transmitted by ingestion of those amyloid fibrils. Characterization of protein species responsible for transmission of mouse AApoAII amyloidosis should provide valuable information. Here, we studied the distributions of ApoA-II and inducing activities in liver fractions that were insoluble or soluble and had different degrees of amyloid deposition. ApoA-II was mainly contained in the 3,000 x g pellet fractions regardless of the degree of amyloid deposition. The 3,000 x g pellet fraction showed strong amyloid fibril-specific fluorescence of fibril-bound thioflavin T and strong amyloidosis-inducing activity. Sonication of liver homogenate increased the proportion of ApoA-II and inducing activity of the 100,000 x g pellet fraction. Weak inducing activity was found in the soluble fraction. We fractionated and isolated multiple assemblies of AApoAII amyloid fibrils by non-denaturing polyacrylamide gel electrophoresis. ApoA-II proteins ranging from monomers to large oligomers had low amyloidosis inducing activity. These results suggest that transmission of AApoAII amyloidosis may be primarily associated with the insoluble amyloid fibril structure.
Purpose : We reviewed our experience of the Kasai operation for biliary atresia (BA) to assess the surgical outcomes. Methods : From May 1993 to May 2013, 58 patients with BA underwent the Kasai operation at our institution. They were divided into two groups, group 1 representing the early period (from 1993 to 2000 ; n＝24) and group 2 the late period (from 2001 to 2013 ; n＝34), based on the difference in postoperative management protocols. Medical records were retrospectively analyzed. Results : There was no statistically significant difference between the two groups in age, body weight, and the serum bilirubin level at operation. The operation time in group 2 was significantly longer than in group 1. The postoperative jaundice-free ratio (PJFR) in group 2 was significantly higher than in group 1 (79.4% vs. 29.2%). There was no significant difference in the PJFR between patients who underwent the Kasai operation after 61 days or more and those before 61 days. The overall 5-year survival rate of the native liver (SRNL) was 37.8%. The SRNL in group 2 was higher than in group 1 (50.0% vs. 26.1%). Conclusions : Improvement in PJFR in group 2 was probably related to changes in postoperative management protocols. Based on our data, early diagnosis and early Kasai operation does not necessarily improve postoperative outcomes such as SRNL or PJFR in patients with BA. The 5-year SRNL in group 2 was higher than in group 1, which might be a result of improvement in PJFR.
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