000 | 03850nam a22005055i 4500 | ||
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001 | 978-94-007-7893-1 | ||
003 | DE-He213 | ||
005 | 20160405110648.0 | ||
007 | cr nn 008mamaa | ||
008 | 140117s2014 ne | s |||| 0|eng d | ||
020 |
_a9789400778931 _9978-94-007-7893-1 |
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024 | 7 |
_a10.1007/978-94-007-7893-1 _2doi |
|
050 | 4 | _aRB155-155.8 | |
050 | 4 | _aQH431 | |
072 | 7 |
_aMFN _2bicssc |
|
072 | 7 |
_aMED107000 _2bisacsh |
|
082 | 0 | 4 |
_a611.01816 _223 |
082 | 0 | 4 |
_a599.935 _223 |
245 | 1 | 0 |
_aProgress in Heritable Soft Connective Tissue Diseases _h[electronic resource] / _cedited by Jaroslava Halper. |
264 | 1 |
_aDordrecht : _bSpringer Netherlands : _bImprint: Springer, _c2014. |
|
300 |
_aVIII, 245 p. 35 illus., 31 illus. in color. _bonline resource. |
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336 |
_atext _btxt _2rdacontent |
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337 |
_acomputer _bc _2rdamedia |
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338 |
_aonline resource _bcr _2rdacarrier |
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347 |
_atext file _bPDF _2rda |
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490 | 1 |
_aAdvances in Experimental Medicine and Biology, _x0065-2598 ; _v802 |
|
505 | 0 | _aStructure, physiology and biochemistry of collagens -- Basic components of connective tissues and extracellular matrix -- Proteoglycans and diseases of soft tissues -- Advances in the use of growth factors for treatment of disorders of soft tissues -- Clinical, diagnostic, and therapeutic aspects of the Marfan syndrome -- Loeys-Dietz syndrome -- Connective Tissue Disorders and Cardiovascular Complications: The indomitable role of Transforming Growth Factor-beta signaling -- The Ehlers-Danlos Syndrome -- Ehlers–Danlos syndrome associated with glycosaminoglycan abnormalities -- Cutis laxa -- Collagen type VI myopathies -- Mouse Models in Tendon and Ligament Research -- Connective tissue disorders in domestic animals. | |
520 | _aThis volume is a reference handbook focusing on diseases like Marfan syndrome, Ehlers-Danlos syndrome, Loeys-Dietz syndrome and other heritable soft connective tissue diseases. The book presents detailed information for both basic scientists and for clinicians seeing patients. It is also a stepping stone for new investigations and studies that goes beyond the facts about the composition and biochemistry of the connective tissue and extracellular matrix, as the authors connect individual components to specific aspects of various soft tissue disorders and to the actual or potential treatment of them. Progress in Heritable Soft Connective Tissue Diseases features very prominent physicians and scientists as contributors who bring their most recent discoveries to the benefit of readers. Their expertise will help clinicians with proper diagnosis of sometimes elusive and uncommon heritable diseases of soft connective tissues. This book also offers an update on the pathophysiology of these diseases, including an emphasis on unifying aspects such as connections between embryonic development of the different types of connective tissues and systems, and the role of TGF-beta in development and physiology of soft tissues. This new set of data explains, at least in part, why many of these disorders are interconnected, though the primary pathophysiological events, such as gene mutations, may be different for each disorder. | ||
650 | 0 | _aMedicine. | |
650 | 0 | _aHuman genetics. | |
650 | 0 | _aInternal medicine. | |
650 | 0 | _aCell physiology. | |
650 | 1 | 4 | _aBiomedicine. |
650 | 2 | 4 | _aHuman Genetics. |
650 | 2 | 4 | _aCell Physiology. |
650 | 2 | 4 | _aInternal Medicine. |
700 | 1 |
_aHalper, Jaroslava. _eeditor. |
|
710 | 2 | _aSpringerLink (Online service) | |
773 | 0 | _tSpringer eBooks | |
776 | 0 | 8 |
_iPrinted edition: _z9789400778924 |
830 | 0 |
_aAdvances in Experimental Medicine and Biology, _x0065-2598 ; _v802 |
|
856 | 4 | 0 | _uhttp://dx.doi.org/10.1007/978-94-007-7893-1 |
912 | _aZDB-2-SBL | ||
999 |
_c3159 _d3159 |