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[CD95(APO-1/Fas)突变在淋巴增殖性疾病和恶性淋巴疾病中的作用]

[The role of CD95 (APO-1/Fas) mutations in lymphoproliferative and malignant lymphatic diseases].

作者信息

Beltinger C, Böhler T, Schrappe M, Ludwig W D, Debatin K M

机构信息

Universitäts-Kinderklinik Ulm, Germany.

出版信息

Klin Padiatr. 1998 Jul-Aug;210(4):153-8. doi: 10.1055/s-2008-1043871.

Abstract

CD95 (APO-1/Fas)-mediated apoptosis plays a major role in normal lymphocyte regulation. CD95 mutations cause a benign autoimmune lymphoproliferation syndrome (ALPS) in mice and humans. CD95 is mutated in some de novo T-lineage acute lymphoblastic leukemia of childhood and these mutations might be of biological significance. The resistance toward CD95-mediated apoptosis observed in most B-lineage ALL is not caused by mutations of CD95. CD95 mutations have been associated with Hodgkin's and Non-Hodgkin's lymphoma and have been described in multiple myeloma. The relevance of CD95 mutations for chemoresistance of ALL requires further study.

摘要

CD95(APO-1/Fas)介导的细胞凋亡在正常淋巴细胞调节中起主要作用。CD95突变在小鼠和人类中导致一种良性自身免疫性淋巴细胞增生综合征(ALPS)。在一些儿童新发T系急性淋巴细胞白血病中CD95发生突变,这些突变可能具有生物学意义。在大多数B系急性淋巴细胞白血病中观察到的对CD95介导的细胞凋亡的抗性并非由CD95突变引起。CD95突变与霍奇金淋巴瘤和非霍奇金淋巴瘤有关,并且在多发性骨髓瘤中也有描述。CD95突变对急性淋巴细胞白血病化疗耐药性的相关性需要进一步研究。

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