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CBA/J小鼠的外分泌性胰腺功能不全综合征。III. 病理和基因分析。

Exocrine pancreatic insufficiency syndrome in CBA/J mice. III. Pathological and genetic analysis.

作者信息

Leiter E H, Cunliffe-Beamer T

出版信息

Gastroenterology. 1977 Aug;73(2):260-6.

PMID:873127
Abstract

A clinical and histopathological description of exocrine pancreatic insufficiency syndrome in CBA/J mice is presented. Amelioration of clinical symptoms by pancreatic enzyme replacement therapy is described, as are breeding experiments designed to test the hypothesis that the etiology of this syndrome is a recessive mutation. Our failure to propagate the expected numbers of animals with the syndrome by genetic means leads us to the conclusion that it is not transmitted as an autosomal recessive mutation. Our finding of focal histological lesions of the exocrine pancreas in large numbers of clinically normal CBA/J mice suggests that the CBA/J genetic background is some way predisposes this strain to the spontaneous necrosis and atrophy of exocrine cells that is pathognomonic of this disease.

摘要

本文介绍了CBA/J小鼠外分泌性胰腺功能不全综合征的临床和组织病理学特征。文中描述了通过胰腺酶替代疗法改善临床症状的情况,以及旨在检验该综合征病因是隐性突变这一假设的繁殖实验。我们未能通过遗传手段繁育出预期数量的患有该综合征的动物,这使我们得出结论,该综合征并非以常染色体隐性突变的方式遗传。我们在大量临床正常的CBA/J小鼠中发现外分泌胰腺存在局灶性组织学病变,这表明CBA/J遗传背景在某种程度上使该品系易患外分泌细胞的自发性坏死和萎缩,而这正是该疾病的特征性表现。

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