BCL-2-DEFICIENT MICE DEMONSTRATE FULMINANT LYMPHOID APOPTOSIS, POLYCYSTIC KIDNEYS, AND HYPOPIGMENTED HAIR
BCL-2-DEFICIENT MICE DEMONSTRATE FULMINANT LYMPHOID APOPTOSIS, POLYCYSTIC KIDNEYS, AND HYPOPIGMENTED HAIR
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DOI：
10.1016/0092-8674(93)80065-m
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发表时间：
1993-10-22
期刊：
影响因子：
64.5
通讯作者：
KORSMEYER, SJ
中科院分区：
文献类型：
--
作者：
VEIS, DJ;SORENSON, CM;KORSMEYER, SJ
bcl-2 -/-小鼠完成胚胎发育，但出生后表现出生长迟缓和早期死亡。造血包括淋巴细胞分化最初是正常的，但胸腺和脾脏经历大量的凋亡退化。胸腺细胞需要凋亡信号来表现加速的细胞死亡。肾功能衰竭是由严重的多囊肾病引起的，其特征是近端和远端肾小管段扩张以及上皮和肾小管过度增殖。bcl-2 -/-小鼠在第二个毛囊周期变灰，暗示氧化还原调节的黑色素合成缺陷。这些功能丧失小鼠的异常表明Bcl-2是一种在抗氧化途径中起作用的死亡阻遏分子。
bcl-2 -/- mice complete embryonic development, but display growth retardation and early mortality postnatally. Hematopoiesis including lymphocyte differentiation is initially normal, but thymus and spleen undergo massive apoptotic involution. Thymocytes require an apoptotic signal to manifest accelerated cell death. Renal failure results from severe polycystic kidney disease characterized by dilated proximal and distal tubular segments and hyperproliferation of epithelium and interstitium. bcl-2 -/- mice turn gray with the second hair follicle cycle, implicating a defect in redox-regulated melanin synthesis. The abnormalities in these loss of function mice argue that Bcl-2 is a death repressor molecule functioning in an antioxidant pathway.