Reproductive Pathology and Sperm Physiology in Acid Sphingomyelinase-Deficient Mice
نویسندگان
چکیده
منابع مشابه
Acid Sphingomyelinase–Deficient Human Lymphoblasts and Mice Are Defective in Radiation-Induced Apoptosis
Stress is believed to activate sphingomyelinase to generate ceramide, which serves as a second messenger in initiating the apoptotic response. Conclusive evidence for this paradigm, however, is lacking. In the present study, we used a genetic approach to address this issue directly. We show that lymphoblasts from Niemann-Pick patients, which have an inherited deficiency of acid sphingomyelinase...
متن کاملSevere impairment in early host defense against Listeria monocytogenes in mice deficient in acid sphingomyelinase.
The phagolysosomal compartment is crucial for the defense against infection with intracellular pathogens. Within this compartment, the TNF- and IFN-gamma-responsive acid sphingomyelinase (ASMase) generates the signaling molecule ceramide, resulting in the activation of proteases like cathepsin D. To investigate the possible role of ASMase as a mediator of the antibacterial effects of TNF and IF...
متن کاملEndoplasmic Reticulum Stress and Homeostasis in Reproductive Physiology and Pathology
The endoplasmic reticulum (ER), comprises 60% of the total cell membrane and interacts directly or indirectly with several cell organelles i.e., Golgi bodies, mitochondria and proteasomes. The ER is usually associated with large numbers of attached ribosomes. During evolution, ER developed as the specific cellular site of synthesis, folding, modification and trafficking of secretory and cell-su...
متن کاملAcid sphingomyelinase-deficient macrophages have defective cholesterol trafficking and efflux.
Cholesterol efflux from macrophage foam cells, a key step in reverse cholesterol transport, requires trafficking of cholesterol from intracellular sites to the plasma membrane. Sphingomyelin is a cholesterol-binding molecule that transiently exists with cholesterol in endosomes and lysosomes but is rapidly hydrolyzed by lysosomal sphingomyelinase (L-SMase), a product of the acid sphingomyelinas...
متن کاملAcid sphingomyelinase-deficient mice mimic the neurovisceral form of human lysosomal storage disease (Niemann-Pick disease)
We have generated an acid sphingomyelinase (aSMase)-deficient mouse line by gene targeting. This novel strain of mutant mouse mimics the lethal, neurovisceral form of the human sphingomyelin storage disease, known as Niemann-Pick disease. Homozygous mice accumulate sphingomyelin extensively in the reticuloendothelial system of liver, spleen, bone marrow, and lung, and in the brain. Most strikin...
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ژورنال
عنوان ژورنال: The American Journal of Pathology
سال: 2002
ISSN: 0002-9440
DOI: 10.1016/s0002-9440(10)64267-8