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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
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.
336 _atext
_btxt
_2rdacontent
337 _acomputer
_bc
_2rdamedia
338 _aonline resource
_bcr
_2rdacarrier
347 _atext file
_bPDF
_2rda
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