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Transthyretin (TTR) is a homotetrameric protein that must misfold in order to form amyloid fibrils. Misfolding includes rate limiting tetramer dissociation, followed by fast tertiary structural changes of the monomer that enable aggregation. Hereditary ATTR amyloidosis is an autosomal dominant genetic disorder with systemic deposition of amyloid fibrils induced by TTR gene mutation. We identified a rare Y114H (p.Y134H) TTR variant in a Japanese patient presenting with late-onset, very mild clinical course. The patient had an extremely low serum variant TTR concentration (18% of total TTR), whereas the composition of variant TTR was 55% in amyloid fibrils in tenosynovial tissues obtained at carpal tunnel release surgery. The amyloid fibril deposits in the ATTR Y114H patient had an altered structure compared with that in wild-type ATTR patients, as determined by luminescent conjugated poly/oligo-thiophene fluorescence spectroscopy. Biophysical studies using recombinant protein showed that Y114H TTR was markedly destabilized both thermodynamically and kinetically and was highly amyloidogenic in vitro. These data suggest that extremely low serum variant Y114H TTR concentration, probably due to endoplasmic reticulum-associated degradation of unstable variant TTR protein, protected this patient from severe amyloidosis, as self-assembly of the amyloidogenic intermediate is a concentration-dependent process. [ABSTRACT FROM AUTHOR]

Analyzes the resiliency that Japan's traditional religion has displayed since the pre-war days despite major economic and political changes. Japanese Supreme Court cases involving religious freedom; Legal and social antecedents for the Supreme Court decision on a suit concerning the enshrinement of the soul of a dead soldier; Tenets of Shinto tradition; Sociological analysis of the code of Japanese culture.

This paper attempts to interpret Japanese institutional syncretism in light of the Weberian theory of rationalization. Much of the historical evidence revolves about the codification of Japanese law and the increasing formalization ( e.g., bureaucratization) of both the political state and religion in early Japan, and particularly in reaction to the influence of China. Weber's differentiation of the types of rationality have enabled us to interpret the occurrence of the patterning of Japanese religion as syncretistic religion. A particular substantive rationality with an emphasis on. methodical ways of life--with the Japanese emperor (tenno) and Shinto rituals as the sacred spiritual foundation, especially among aristocracies--subjugated practical rationality and led to the development of formal rationality in early Japan. [ABSTRACT FROM AUTHOR]

The article focuses on adaptation and resistance to Chinese literary hegemony in Korea and Japan. China was the first civilization to emerge in East Asia. Accordingly, when neighboring nations such as the Koreans, the Vietnamese and the Japanese began their own climb from prehistorical archaism toward civilization, they inevitably looked towards China as the ancient fount of culture and to a large extent borrowed its institutions and inventions. Written Chinese played a vital role in East Asia, similar to that of Latin in Europe, as the language of education between the 3rd and 9th centuries. Chinese because established as the common language of East Asia. In Korea, Japan, and elsewhere, written Chinese became the official language of state documents. The reason for this was straightforward, the Chinese court's power dominated the East Asian nations. By using a theory of the effect of literacy taken from social anthropology and sociology, this paper explores the social implications of kana literacy in ancient and early medieval Japan. This paper, however, does not posit literacy as an effective cause independent of the social and political environment, as do some leading theoretical models. Rather, it assumes that the implications of literacy for power arrangements in general in a particular cultural context are likely to be closely connected to who has power to control that literacy, what purposes it serves, and in what contexts it is utilized.

This paper explores some dynamic aspects of the religio-political problems in Japan as expressions of the revitalization movement of modem Japanese civil religion, of which state Shinto is an important part. Many governmental leaders are convinced that Japan needs to restore its sacred Shinto symbols that once gave the people of Japan a strong national identity and cosmic unity. This conviction is expressed in political and religious leaders' persistent efforts to again make the Yasukuni Shrine the national shrine. It is also seen in their efforts to revise social studies and history textbooks for the patriotic education of children. The paper attempts to identify some of the cultural, historical and economic reasons for the revitalization movement and the dilemma it presents. [ABSTRACT FROM AUTHOR]