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68 min
1. The mononuclear phagocyte system: tissue resident macrophages - activation and regulation- Prof. Emeritus Siamon Gordon
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25 min
2. Biosimilars: when value meets cost- Prof. Fernando de Mora
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25 min
3. Mononuclear phagocytes - origins, fates and functions- Prof. Steffen Jung
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39 min
4. Transcription factors in macrophage differentiation- Dr. Michael Sieweke
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44 min
5. Fetal macrophages- Prof. Paul Martin
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44 min
6. The alveolar macrophage- Dr. Ronald G. Crystal
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35 min
7. Airway macrophages in health and disease- Prof. Tracy Hussell
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65 min
8. Intestinal macrophages - heterogeneity, origins and functions- Prof. Allan Mowat
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27 min
9. Osteoclasts: what do they do and how do they do it?- Prof. Steven L. Teitelbaum
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51 min
10. The biology of Fc receptors and complement receptors- Prof. Steven Greenberg
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71 min
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38 min
12. Tumour-associated macrophages- Prof. Michele De Palma
- Dr. Mario Squadrito
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42 min
13. The recognition of pathogens by C-type lectins- Prof. Gordon D. Brown
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34 min
14. Macrophage phagocytosis- Prof. Joel Swanson
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30 min
15. Macrophage CD36 and atherosclerosis- Dr. Maria Febbraio
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31 min
16. Toll-Like receptor signaling and the innate immune response- Dr. Kate Fitzgerald
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43 min
17. Innate immune receptors as mediators of systemic inflammation and pathogenesis of malaria- Prof. Ricardo Gazzinelli
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34 min
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69 min
19. Triggering receptors expressed on myeloid cells (TREM)- Dr. Daniel W. McVicar
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60 min
20. Influence of eicosanoid lipid mediators on macrophage innate immune functions- Prof. Marc Peters-Golden
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47 min
21. Macrophage paired receptor interactions- Prof. Neil Barclay
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45 min
22. Macrophage heterogeneity in atherosclerosis regression- Prof. Edward Fisher
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35 min
23. Gaucher disease: from lysosomal storage to immunopathology- Prof. Johannes M.F.G. Aerts
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26 min
24. Macrophage in asthma- Prof. Douglas Robinson
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51 min
25. The macrophage mannose receptor- Dr. Luisa Martinez-Pomares
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41 min
26. Myeloid-derived suppressor cells in cancer- Prof. Dmitry Gabrilovich
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29 min
27. EGF-TM7 receptors- Dr. Jörg Hamann
- Dr. Hsi-Hsien Lin
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37 min
28. Macrophages in helminth infection- Prof. Judith Allen
- Archived Lectures *These may not cover the latest advances in the field
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49 min
29. Immunosuppressive mechanisms in myeloid cells- Prof. Dmitry Gabrilovich
Printable Handouts
Navigable Slide Index
- Introduction
- Speaker's personal background
- Clinical expression of Gaucher disease (GD)
- Molecular basis of Gaucher disease
- Enzyme deficiency & lysosomal lipid accumulation
- Type 1 Gaucher disease
- Genetic & phenotypic heterogeneity
- Discordant monozygotic Gaucher twins
- Sources of GlcCer substrate
- Gaucher cells driving visceral pathology
- Is type 1 GD only a disorder of macrophages?
- Disease; storage cells; therapeutic correction
- Therapies for type 1 Gaucher disease
- Enzyme replacement therapy
- Disease; storage cells; biomarkers
- Protein biomarkers
- Chitotriosidase: a marker for Gaucher disease
- Gaucher cells as source of chitotriosidase
- Chitotriosidase: the human macrophage chitinase
- Plasma chitotriosidase changes upon ERT
- Use in clinical management of type 1 GD patients
- Chitotriosidase plasma levels
- Pitfalls with chitotriosidase measurement
- Superior substrate for chitotriosidase assay
- Chitotriosidase deficiency
- Chemokine CCL18/PARC
- Elevated CCL18 levels in Gaucher serum
- Protein composition of Gaucher cells: proteomics
- Galectin-3: newly discovered protein abnormality
- Markers of skeletal disease in Gaucher patients
- MIP-1alpha and MIP-1beta in GD plasma
- Gaucher cells don't produce MIP-1beta or alpha
- Plasma MIP-1beta and skeletal disease
- Potential lipid biomarkers
- Increased plasma GlcCer and GM3 in type 1 GD
- Increased plasma GlcSph in type 1 GD
- Plasma GlcSph correction by ERT
- Plasma GlcSph and disease manifestations
- Glucosylsphingosine: a cytotoxic compound
- Plasma GlcSph: a Gaucher biomarker
- Other pathologies related to Gaucher disease
- Skin pathophysiology
- Brain pathophysiology: different hypotheses
- Bone disease
- Osteoporosis in Gaucher disease
- Metabolic abnormalities
- Impaired insulin sensitivity
- Growth retardation
- Increased risk for cancer
- Increased risk for parkinsonism
- Immune system: the missing link?
- Acknowledgements
Topics Covered
- Gaucher disease
- Clinical manifestations
- Primary defect: gene, enzyme, storage material
- Visceral pathology: organomegaly and haematological complications
- Gaucher cells: lipid-laden macrophages
- Circulating markers for storage cells
- Therapy
- Other pathological aspects of Gaucher disease: bone, brain, body metabolism
Links
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Talk Citation
Aerts, J.M. (2013, January 30). Gaucher disease: from lysosomal storage to immunopathology [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved April 19, 2025, from https://doi.org/10.69645/SCCV5128.Export Citation (RIS)
Publication History
- Published on January 30, 2013
Financial Disclosures
- Prof. Johannes M.F.G. Aerts has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.
Gaucher disease: from lysosomal storage to immunopathology
Published on January 30, 2013
35 min
