异质性细胞器幻灯片.ppt
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1、异质性细胞器第1页,共10页,编辑于2022年,星期六Characteristics of LysosomesLysosome is a Lysosome is a heterogenous heterogenous organelleorganelle(异质性(异质性(异质性(异质性细胞器)细胞器)细胞器)细胞器):l lPrimary Primary lysosomelysosome(初级溶(初级溶(初级溶(初级溶酶体)酶体)酶体)酶体)l lSecond lysosomesSecond lysosomes(次级溶酶体)(次级溶酶体)(次级溶酶体)(次级溶酶体)l l heterophag
2、icheterophagic(异噬溶酶体)(异噬溶酶体)(异噬溶酶体)(异噬溶酶体)l l autophagicautophagic(自噬溶酶体)(自噬溶酶体)(自噬溶酶体)(自噬溶酶体)l lResidual bodyResidual body(残余小体)(残余小体)(残余小体)(残余小体)Primary LysSecond Lys第2页,共10页,编辑于2022年,星期六The Functions of LysosomesvvLysosomes are involved Lysosomes are involved in three major cell in three major ce
3、ll functions:functions:phagocytosis phagocytosis(吞噬)(吞噬)(吞噬)(吞噬)A summary of the phagocytic pathway第3页,共10页,编辑于2022年,星期六 autophagy(自吞)(自吞)Electron micrograph of a mitochondrion and peroxisome(过氧化物酶体)enclosed in a double membrane wrapper derived from the ER.This autophagic vacuole would have fused wi
4、th a lysosome and its contents digested.第4页,共10页,编辑于2022年,星期六 endocytosis(内吞(内吞作用)作用)第5页,共10页,编辑于2022年,星期六Disorders Resulting from Defects in lysosomal FunctionDisorders Resulting from Defects in lysosomal Function I-cell disease:Many cells in these patients contain lysosomes that are bloated with u
5、ndergraded materials.When fibroblasts from these patients were studied in culture,it was found that lysosomal enzymes are synthesized at normal levels but are secreted into the medium and not targeted to lysosomes.第6页,共10页,编辑于2022年,星期六The I-cell defect was soon traced to the deficiency of an enzyme(
6、N-acetyglucosamine phosphotransferase)required for mannose phosphorylation.The secreted enzymes lacked the mannose phosphate residue that are present on the corresponding enzymes of cells from normal individuals.第7页,共10页,编辑于2022年,星期六Pompe disease:a fatal inherited conditiona fatal inherited conditio
7、n in the absence of-glucosidase,undigested glycogen in the absence of-glucosidase,undigested glycogen accumulated in lysosomes,causing swelling of the organelles accumulated in lysosomes,causing swelling of the organelles and irreversible damage to the cells and tissuesand irreversible damage to the
8、 cells and tissuesDiseases of this type,characterized by the deficiency of a single Diseases of this type,characterized by the deficiency of a single lysosomal enzyme and the corresponding accumulation of lysosomal enzyme and the corresponding accumulation of undergraded substrate,are called undergr
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