最新异质性细胞器PPT课件.ppt

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1、异质性细胞器异质性细胞器Characteristics of LysosomesLysosome is a Lysosome is a heterogenous heterogenous organelleorganelle（异质（异质（异质（异质性细胞器）性细胞器）性细胞器）性细胞器）:1.1.Primary Primary lysosomelysosome（初级（初级（初级（初级溶酶体）溶酶体）溶酶体）溶酶体）2.2.Second Second lysosomeslysosomes（次（次（次（次级溶酶体）级溶酶体）级溶酶体）级溶酶体）3.3.heterophagicheterophagi

2、c4.4.（异噬溶酶体）（异噬溶酶体）（异噬溶酶体）（异噬溶酶体）5.5.autophagicautophagic6.6.（自噬溶酶体）（自噬溶酶体）（自噬溶酶体）（自噬溶酶体）7.7.Residual bodyResidual body8.8.（残余小体）（残余小体）（残余小体）（残余小体）Primary LysSecond LysOther lysosomal storage diseasesDiseaseDiseaseEnzyme Enzyme DeficiencyDeficiencyPrinciple Storage Principle Storage substancesubstan

3、ceconsequencesconsequencesGGM1M1 Gangliosid-Gangliosid-osisosisGM1-GM1-GalactosidaseGalactosidaseGanglioside GGanglioside GM1M1Mental retardation,liver Mental retardation,liver enlargement,skeletal enlargement,skeletal involvement,death by age 2 involvement,death by age 2 Tay-Sachs Tay-Sachs dieased

4、ieaseHexosaminidasHexosaminidase Ae AGanglioside GGanglioside GM2M2Mental retardation,blindness,death Mental retardation,blindness,death by age 3by age 3Fabrys Fabrys diseasedisease-Galactosidase Galactosidase A ATrihexosylceramiTrihexosylceramidedeSkin rash,kidney failure,pain in Skin rash,kidney f

5、ailure,pain in lower extremitieslower extremitiesSandhoffsdisSandhoffsdiseaseeaseHexosaminidasHexosaminidase A and Be A and BGanglioside GGanglioside GM2 M2 and globosideand globosideSimilar to Tay-Sachs diease but Similar to Tay-Sachs diease but more rapidly progressingmore rapidly progressingGauch

6、ers Gauchers diseasediseaseGlucocerebrosiGlucocerebrosidasedaseGlucocerebrosideGlucocerebrosideLiver and spleen Liver and spleen enlargement,erosion of long enlargement,erosion of long bones,mental retardation in bones,mental retardation in infantile form onlyinfantile form onlyTay-Sachs disease:the

7、 best studied lysosomal the best studied lysosomal storage diseasesstorage diseases It results from a deficiency of the enzyme-N-It results from a deficiency of the enzyme-N-hexosaminidase A,an enzyme that degrades the ganglioside hexosaminidase A,an enzyme that degrades the ganglioside GGM2M2 G GM2

8、M2 is a major component of the membranes of brain cells is a major component of the membranes of brain cells,and in the absence of the hydrolytic enzyme,the ganglioside,and in the absence of the hydrolytic enzyme,the ganglioside accumulates in the bloated lysosomes of brain cells,causing accumulates

9、 in the bloated lysosomes of brain cells,causing dysfunctiondysfunction In its severe form,which strikes during infancy,the disease In its severe form,which strikes during infancy,the disease is chacterized by progressive mental and motor retardation,is chacterized by progressive mental and motor retardation,as well as skeletal,cardiac,and resiratory abnormalitiesas well as skeletal,cardiac,and resiratory abnormalities