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重症联合免疫缺陷病中的平行管状阵列:外周血淋巴细胞的超微结构研究

Parallel tubular arrays in severe combined immunodeficiency disease: an ultrastructural study of peripheral blood lymphocytes.

作者信息

Payne C M, Jones J F, Sieber O F, Fulginiti V A

出版信息

Blood. 1977 Jul;50(1):55-64.

PMID:194640
Abstract

The ultrastructure of the lymphocytes from three children with severe combined immunodeficiency disease (SCID) is described. Parallel tubular arrays (PTA) were found in a large percentage of circulating lymphocytes (53%, 41%, and 13%) in three SCID patients when compared to age-matched controls. The size of these inclusions was quite variable, with some attaining a length of 1.7 micrometer. They contained a tubular substructure with a diameter of 36--44 nm. The PTA were mostly located in the centriolar and Golgi regions of the cytoplasm, and were sometimes membrane bound. A centriolar origin of the inclusion was suggested. A second inclusion, the tubuloreticular structure, was found in only 1.4% of the circulating lymphocytes from one SCID patient. The origin of the PTA and its occurrence in severe combined immunodeficiency disease are discussed.

摘要

本文描述了三名患有严重联合免疫缺陷病(SCID)儿童淋巴细胞的超微结构。与年龄匹配的对照组相比,在三名SCID患者中,很大比例的循环淋巴细胞(分别为53%、41%和13%)中发现了平行管状阵列(PTA)。这些包涵体的大小差异很大,有些长度可达1.7微米。它们含有直径为36 - 44纳米的管状亚结构。PTA大多位于细胞质的中心粒和高尔基体区域,有时有膜包被。提示该包涵体起源于中心粒。在一名SCID患者的循环淋巴细胞中,仅1.4%发现了另一种包涵体——小管状网状结构。本文讨论了PTA的起源及其在严重联合免疫缺陷病中的出现情况。

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