Bcl-2-deficient mice demonstrate fulminant lymphoid apoptosis, polycystic kidneys, and hypopigmented hair

作者: Deborah J. Veis , Christine M. Sorenson , John R. Shutter , Stanley J. Korsmeyer

DOI: 10.1016/0092-8674(93)80065-M

关键词: BiologyLymphocyte differentiationPolycystic kidney diseaseInvolution (medicine)PathologyEndocrinologyHair follicleProgrammed cell deathApoptosisInternal medicineSpleenLymphatic system

摘要: bcl-2-/-mice complete embryonic development, but display growth retardation and early mortality postnatally. Hematopoiesis including lymphocyte differentiation is initially normal, thymus spleen undergo massive apoptotic involution. Thymocytes require an signal to manifest accelerated cell death. Renal failure results from severe polycystic kidney disease characterized by dilated proximal distal tubular segments hyperproliferation of epithelium interstitium. turn gray with the second hair follicle cycle, implicating a defect in redox-regulated melanin synthesis. The abnormalities these loss function mice argue that Bcl-2 death repressor molecule functioning antioxidant pathway.

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