细胞器与疾病.ppt
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1、细胞器与疾病 (分子医学),A mammalian cell,Nucleus,The nucleus is a membrane bound structure that contains the cells hereditary information and controls the cells growth and reproduction. It is commonly the most prominent organelle in the cell,Nuclear Membranes,The nuclear content is located in the nuclear lume
2、n and is surrounded by a double membrane or nuclear envelope, composed of inner membrane and outer membrane. The outer membrane is contiguous with the ER The nuclear membrane contains nuclear pores, which provide selective access into and out of the nuclear lumen The inner membrane has a protein lin
3、ing called the nuclear lamina, which binds to chromatin and other nuclear components.,The Nuclear Pore Complexes form a continuous aqueous channel between cytoplasm & nucleoplasm,- nuclear pore complex is large about 120 million Daltons -30 different proteins found in pore; basic subunit repeated 16
4、 times. “Central granule“ now called “transporter“ functions to move molecules through pore. There is a ring of proteins that anchors pore to N.E. and the “basket“ of fibers with unknown function no obvious motor proteins were found,The Nucleolus,The region of the nucleus where portions of chromosom
5、es that contain genes coding for ribosomal RNA are transcribed and ribosomal subunits are assembled,http:/ of DNA with rRNA genes nucleolar organizing region = (NOR) Ribosomal proteins are synthesized in cytoplasm and transported into the nucleus These proteins self associate with appropriate rRNA d
6、uring rRNA synthesis forming immature ribosomal subunits Ribosomes finish self assembly in cytoplasm,Endoplasmic Reticulum,KEDLR-GFP,激光共聚焦显微镜下的ER,The Endoplasmin Reticulum (ER) is an extensive, extra-nuclear membrane system with the following functions:,ER is a home for various enzymes involved in p
7、rotein folding , drug detoxification, membrane lipid biosynthesis, cholesterol and fatty acid metabolism ER is an entry point for protein sorting. Targeting of these proteins is mediated by signal sequence. The membrane proteins are inserted into the ER membrane in their proper orientation. Secreted
8、 proteins are translocated into ER lumen and then transported to the destination place,Rough and Smooth ER,Two regions of the ER differ in both structure and function. Rough ER has ribosomes attached to the cytoplasmic side of the membrane. Smooth ER lacks attached ribosomes. Typically, the smooth E
9、R is a tubule network and the rough ER is a series of flattened sacs.,http:/esg-www.mit.edu:8001/esgbio/cb/org/er.gif,The smooth ER has a wide range of functions including carbohydrate and lipid synthesis. It serves as a transitional area for vesicles that transport ER products to various destinatio
10、ns. In liver cells the smooth ER produces enzymes that help to detoxify certain compounds. In muscles the smooth ER assists in the contraction of muscle cells and in brain cells it synthesizes male and female hormones.,The rough ER manufactures membranes and secretory proteins,The rough and smooth E
11、R are Usually interconnected and the proteins and membranes made by the rough ER move into the smooth ER to be transferred to other locations.,The cytoplasm has a reducing environment, while ER lumen is oxidizing . This difference is generated by unequal distribution of trypeptide glutathione and is
12、 essential for formation of disulfide bonds in proteins and for proper folding,ER function-related diseases,内质网应激与心血管疾病 内质网与细胞凋亡 内质网与PrP and Mad Cow,Mitochondria,Mitochondria (singular: mitochondrion) are the sites of aerobic respiration, and generally are the major energy production center in eukar
13、yotes,The number of mitochondria range from one to thousands per cells. They are often positioned in cells nearest to sites of energy utilization One of the richest sources of mitochondria is a hummingbird flight muscle,Mitochondria are a double membrane organelle in which the inner membrane is in-f
14、olded to form “cristae”.,The outer membrane is a fairly simple phospholipid bilayer, containing porins, proteins that render it permeable to molecules of about 10 kilodaltons or less. Ions, nutrient molecules, ATP, ADP, etc. easily pass through the outer membrane and enter the intermembrane space Th
15、e inner membrane is more complex and contains respiratory chains and transporters,The matrix lies within the inner membrane. The access to this compartment often requires specific transporters,Four possible localization for mitochondrial enzymes,The Golgi Complex,Located near cell nucleus, consists
16、of flattened, membrane-bounded sacs (cisternae) forming a stack,Each stack has: cis-face is an entry face - adjacent to ER to accept incoming vesicles,trans-face is an exit face points towards plasma membrane, produces vesicles for forward flow,The function of the Golgi is to transport and process s
17、ecreted and membrane proteins from ER to the cell surface,Cis removal of mannose, phosphorylation Medial removal of mannose, addition of N-acetylglucosamine Trans Removal of galactose, addition of sialic acid TGN addition of sialic acid, Sorting,Cisternae segregated into convex (“cis“), medial (midd
18、le), and concave (“trans“) compartments.,ER,cys,medial,trans,TGN,Vesicular Transport,Golgi structure/function/-related diseases,Golgi and Mitosis Golgi and Apoptosis Golgi and Liver Cancer,HNE induces Golgi Fragments,Control,30 min,1 h,2 h,4 h,The Endocytic Pathway,Endosomes and Lysosomes,Lysosomes,
19、1、初级溶酶体 直径约0.20.5um膜厚7.5nm,内含物均一,无明显颗粒,是高尔基体分泌形成的(如右图)。含有多种水解酶,但没有活性,只有当溶酶体破裂,或其它物质进入,才有酶活性。其水解酶包括蛋白酶,核酸酶、脂酶、磷酸酶、硫酸酯酶、磷脂酶类,已知60余种,这些酶均属于酸性水解酶,反应的最适PH值为5左右,溶酶体膜虽然与质膜厚度相近,但成分不同,,2、次级溶酶体 这些都是消化泡,正在进行或完成消化作用的溶酶体,内含水解酶和相应的底物,可分为自噬溶酶体(autophagolysosome)和异噬溶酶体(phagolysosome),前者消化的物质来自细胞本身的各种组分,后者消化的物质来自外源。
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