| 1. | |
Introduction | Introduction is given to the entire content of the lecture series, and which books are used and that Refswork can be used to download the slides presented during the lectures. An overview is given about the principal techniques used to study the early secretory pathway and cellular digestive systems. |  |
| 2. | |
Endoplasmic reticulum: Structural domains, domain formation and function, shape-forming proteins | Cell and organ-specific quantitative aspects/differences for the ER are discussed. The ER domains nuclear envelope and rough ER are presented in terms of structure and function as well pathological changes due to protein accumulation and their consequences. | |
| 3. | |
Ilnuma T, et al.: Role of syntaxin 18 in the organization of endoplasmic reticulum subdomains. J Cell Sci 122:1680, 2009Hu J, et al.: Membrane proteins of the endoplasmic reticulum induce high-curvature tubules. Science 319:1247, 2008 | Presentation and discusssion of a scientific publication on the role of syntaxin 18 for rough and smooth endoplasmic reticulum domains. Presentation and discusssion of a scientific publication on form-shaping proteins in the endoplasmic reticulum. | |
| 6. | |
Endoplasmic reticulum:Quality control of protein folding and assembly, protein folding disorders,chemical chaperones, congenital disorders of glycosylation | House keeping function of protein quality control.Quality control of protein folding under conditions of excess of misfolded glycoproteins. Recognition and retention of misfolded glycoproteins in the endoplasmic reticulum and pre Golgi intermediates. Glycocodes for protein folding. Calnexin/calreticulin cycle. Importance of EDEM1 and OS-9 for dislocation. | |
| 7. | |
Endoplasmic reticulum:Quality control of protein folding and assembly, protein folding disorders,chemical chaperones, congenital disorders of glycosylation | Types of protein folding disorders. Definition, classes, properties and use of synthetic chaperones in protein folding disorders. Alpha-galactosidase A competitive inhibitor for Fabry disease. Phenylbutyric acid as chemical chaperone for myocilin-induced glaucoma. Definition and characterization of loss-of-function and pathological gain-of-function folding disorders. | |
| 8. | |
Endoplasmic reticulum:Quality control of protein folding and assembly, protein folding disorders,chemical chaperones, congenital disorders of glycosylation | Dislocation of misfolded glycoproteins by ERAD-L, ERAD-M and ERAD-C. Molecular machines generated by dislocation machinery proteins. Structure and function of EDEM 1 and OS9 for protein dislocation. Mechanism of ubiquitination and dislocation of misfolded glycoproteins. Proteasomal and autophagic degradation. Diseases of glycosylation with emphasis on molecular basis , diagnostic and therapy of congenital diseases of glycosylation. | |
| 9. | |
Transitional endoplasmic reticulum and pre-Golgi intermediates: COP proteins, ER - Golgi transport, COP protein disorders | Structure of pre-Golgi intermediates and transport by microtubules. Function of pre-Golgi intermediates in vesicular transport from ER to Golgi apparatus. Transitional elements of the ER and formation of COPII coat and COPII vesicles. | |
| 10. | |
Golgi apparatus: structure, vesicular transport versus cisternal maturation, protein transport in the Golgi apparatus | Techniques to visualize the Golgi apparatus in cells and tissues. Structural and 3D-organization of the Golgi apparatus. Topography of Golgi compartments. Importance of the cytoskeleton and peripheral proteins for Golgi apparatus structure. | |
| 11. | |
Golgi apparatus: structure, vesicular transport versus cisternal maturation, protein transport in the Golgi apparatus | Formation and assembly of COPI coats and COPI vesicles through Arf1 and COPI coat proteins. COPI vesicle transport in the Golgi apparatus and retrieval to the ER. Mechanisms of intra-Golgi apparatus transport of cargo. | |
| 12. | |
Long, K. R., et al.: Sar1 assembly regulates membrane constriction and ER export. J Cell Biol 190: 115-128, 2010 | Presentation and discussion of a publication on Sar1 involvement in ER-to-Golgi apparatus transport | |
| 13. | |
Friedman, J. R., et al.: ER sliding dynamics and ER-mitochondrial contacts occur on acetylated microtubules. J Cell Biol 190: 363-375, 2010 | Presentation and discussion of a scientific publication on organelle transport along microtubules | |
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