The Extracellular Matrix in Genetic Skeletal Disorders
This book explores how defects in extracellular matrix (ECM) proteins, their post-translational modifications, and intracellular trafficking impact cartilage and bone integrity. It underscores the ECM's role in providing structural support, establishing morphogenetic gradients, and interacting...
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| Andre forfattere: | , |
| Format: | Electronisk eBog |
| Sprog: | engelsk |
| Udgivet: |
Cham :
Springer Nature Switzerland : Imprint: Springer,
2024.
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| Udgivelse: | 1st ed. 2024. |
| Serier: | Biology of Extracellular Matrix,
16 |
| Fag: | |
| Online adgang: | https://doi.org/10.1007/978-3-031-70835-0 |
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| 245 | 1 | 4 | |a The Extracellular Matrix in Genetic Skeletal Disorders |h [electronic resource] / |c edited by Antonio Rossi, Frank Zaucke. |
| 250 | |a 1st ed. 2024. | ||
| 264 | 1 | |a Cham : |b Springer Nature Switzerland : |b Imprint: Springer, |c 2024. | |
| 300 | |a X, 410 p. 31 illus., 29 illus. in color. |b online resource. | ||
| 336 | |a text |b txt |2 rdacontent | ||
| 337 | |a computer |b c |2 rdamedia | ||
| 338 | |a online resource |b cr |2 rdacarrier | ||
| 341 | 0 | |b Table of contents navigation |2 onix | |
| 341 | 0 | |b Single logical reading order |2 onix | |
| 341 | 0 | |b Short alternative textual descriptions |2 onix | |
| 341 | 0 | |b Use of color is not sole means of conveying information |2 onix | |
| 341 | 0 | |b Use of high contrast between text and background color |2 onix | |
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| 341 | 0 | |b All non-decorative content supports reading without sight |2 onix | |
| 347 | |a text file |b PDF |2 rda | ||
| 490 | 1 | |a Biology of Extracellular Matrix, |x 2191-1959 ; |v 16 | |
| 505 | 0 | |a Part I: ECM proteins and skeletal disorders -- Chapter 1: Extracellular matrix and skeletal dysplasias -- Chapter 2: Osteogenesis imperfecta and related diseases – collagen I alterations in skeletal and extraskeletal tissues -- Chapter 3: Bone involvement in the Ehlers-Danlos syndromes -- Chapter 4: Collagen II mutations in inherited cartilage diseases: our current understanding of genotype-phenotype correlations -- Chapter 5: Skeletal disorders linked to GAG synthesis -- Chapter 6: GAG sulfation and skeletal disorders -- Chapter 7: The fibrillinopathies – fibrillin-dependent skeletal dysplasias affecting long bone growth -- Part II: ECM signaling and skeletal disorders -- Chapter 8: FGF signaling: a key pathway during skeletal development -- Chapter 9: Impaired Wnt signaling as a cause of skeletal disorders -- Chapter 10: The skeletal ciliopathies -- Part III: Intracellular trafficking of ECM proteins and related skeletal disorders -- Chapter 11: Microtubule-associated motor proteins in skeletal development and health -- Chapter 12: COG4 variant and Saul-Wilson Syndrome -- 13: Membrane trafficking to the extracellular matrix and skeletal dysplasia. | |
| 520 | |a This book explores how defects in extracellular matrix (ECM) proteins, their post-translational modifications, and intracellular trafficking impact cartilage and bone integrity. It underscores the ECM's role in providing structural support, establishing morphogenetic gradients, and interacting with cell surface receptors in musculoskeletal tissues. The book delves into the structure and biology of the ECM in the skeleton, discussing skeletal disorders caused by mutations in genes associated with ECM proteins, synthesis, turnover, and signal transduction. Authored by experts who have made significant discoveries in the molecular mechanisms of skeletal disorders and are developing therapeutic strategies, this book is an invaluable research for both scientists and clinicians seeking a comprehensive understanding of this growing and exciting field. The series Biology of Extracellular Matrix is published in collaboration with the American Society for Matrix Biology and the International Society for Matrix Biology. | ||
| 532 | 8 | |a Accessibility summary: This PDF does not fully comply with PDF/UA standards, but does feature limited screen reader support, described non-text content (images, graphs), bookmarks for easy navigation and searchable, selectable text. Users of assistive technologies may experience difficulty navigating or interpreting content in this document. We recognize the importance of accessibility, and we welcome queries about accessibility for any of our products. If you have a question or an access need, please get in touch with us at accessibilitysupport@springernature.com. | |
| 532 | 8 | |a No reading system accessibility options actively disabled | |
| 532 | 8 | |a Publisher contact for further accessibility information: accessibilitysupport@springernature.com | |
| 650 | 0 | |a Cytology. | |
| 650 | 0 | |a Musculoskeletal system. | |
| 650 | 0 | |a Clinical biochemistry. | |
| 650 | 0 | |a Diseases. | |
| 650 | 1 | 4 | |a Cell Biology. |
| 650 | 2 | 4 | |a Musculoskeletal system. |
| 650 | 2 | 4 | |a Medical Biochemistry. |
| 650 | 2 | 4 | |a Mechanisms of Disease. |
| 700 | 1 | |a Rossi, Antonio. |e editor. |0 (orcid)0000-0002-2460-8761 |1 https://orcid.org/0000-0002-2460-8761 |4 edt |4 http://id.loc.gov/vocabulary/relators/edt | |
| 700 | 1 | |a Zaucke, Frank. |e editor. |4 edt |4 http://id.loc.gov/vocabulary/relators/edt | |
| 710 | 2 | |a SpringerLink (Online service) | |
| 773 | 0 | |t Springer Nature eBook | |
| 776 | 0 | 8 | |i Printed edition: |z 9783031708343 |
| 776 | 0 | 8 | |i Printed edition: |z 9783031708367 |
| 776 | 0 | 8 | |i Printed edition: |z 9783031708374 |
| 830 | 0 | |a Biology of Extracellular Matrix, |x 2191-1959 ; |v 16 | |
| 856 | 4 | 0 | |u https://doi.org/10.1007/978-3-031-70835-0 |
| 912 | |a ZDB-2-SBL | ||
| 912 | |a ZDB-2-SXB | ||
| 950 | |a Biomedical and Life Sciences (SpringerNature-11642) | ||
| 950 | |a Biomedical and Life Sciences (R0) (SpringerNature-43708) | ||