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Thestructuralproteins
Elastin
Elastin confersextensibilityandelasticrecoilintissues. y
Elastin
Unlikecollagen,thereisonlyonegenetictypeofelastin. g , y g yp
Elastin
Unlikecollagen,elastin doesnotcontainGlyXY sequences,triplehelicalstructure,orcarbohydrate t i l h li l t t b h d t moieties.
Elastin
Similartocollagen,certainlysyl residuesoftropoelastin are y y y yy oxidatively deaminated toaldehydes bylysyl oxidase.
Desmosines crosslink
MajorDifferencesBetweenCollagenandElastin Collagen
Manydifferentgenetictypes Triplehelix Triple helix ( y (GlyXY)nrepeatingstructure ) p g Presenceofhydroxylysine Carbohydratecontaining
Elastin
Onegenetictype Notriplehelix;randomcoil No triple helix; random coil conformationspermittingstretching No(GlyXY)nrepeatingstructure ( y ) p g Nohydroxylysine Nocarbohydrate
Intramolecular aldol crosslinks I t I t l l ld l li k Intramolecular d l l desmosine crosslinks i li k Presenceofextension peptidesduringbiosynthesis Noextensionpeptidespresentduring p p p g biosynthesis
Fibrilin 1 Fibrilin1
Isalargeglycoprotein. Is a large glycoprotein Itisastructuralcomponentofmicrofibrils inmany It is a structural component of microfibrils in many tissues(lens,periosteum,aorta). Issecretedbyfibroblastsandbecomesincorporatedinto theinsolublemicrofibrils. MutationsareassociatedwithMarfan syndrome.
Marfansyndrome
Thespecializedproteins
Fibronectin
Theroleoffibronectins istoattachcellstoavarietyof extracellularmatrices.
Fibronectin
Fibronectin containsanArgGlyAsp(RGD)sequencethat bindstotheintegrin receptors. TheRGDsequenceissharedbyanumberofother proteinsintheECMthatbindtointegrins presentincell surfaces.
Laminins
Thelaminins areafamilyofglycoproteins foundinthe basallamina. Thelaminins areanimportantforcelldifferentiation, migration,adhesion. migration adhesion Laminins are trimeric proteins that contain an chain a aretrimeric proteinsthatcontainanchain,a chain,andachainlinkedtogethertoforman elongatedcruciformshape.. elongated cruciform shape..
Laminin
IthaspotentialbindingsitesfortypeIVcollagen,heparin,and It has potential binding sites for type IV collagen, heparin, and integrins oncellsurfaces.
Theproteoglycans
Proteoglycans
Proteoglycansareproteinsthatcontaincovalentlylinked glycosaminoglycans. Thebasicproteoglycan unitconsistsofa"coreprotein" withoneormorecovalentlyattachedglycosaminoglycan (GAG)chain.
Schematicrepresentationoftheproteoglycan aggrecan.
Proteoglycans
Atleast30typeshavebeencharacterizedandgiven namessuchassyndecan,betaglycan,serglycin,perlecan, aggrecan,versican,decorin,biglycan,andfibromodulin. i d i bi l d fib d li Theyvaryintissuedistribution,natureofthecore protein,attachedglycosaminoglycans,andfunction.
Thefunctionofproteoglycans p gy
1. Bindwaterandformhydratedmatrices. 1 Bi d df h d d i 2. Provideascaffoldforepithelialcellmigration,proliferation, anddifferentiation. 3. Regulatevariousbiologicalactivitiessuchascoagulation, hostdefense,andwoundrepair.
Thefunctionofproteoglycans p gy
4. Bindcytokines,chemokines,growthfactors,and 4 Bi d ki h ki hf d morphogens,protectingthemagainstproteolysis. 5. Actasreceptorsforproteasesandproteaseinhibitors regulatingtheirspatialdistributionandactivity. l ti th i ti l di t ib ti d ti it 6. Membraneproteoglycanscanactasendocytic receptors forclearanceofboundligands.
Glycosaminoglycans
Glycosaminoglycans
AGAGisanunbranched polysaccharidemadeupof repeatingdisaccharides. Onecomponentofthedisaccharideisalwaysan aminosugar,eitherDglucosamineorD g , g galactosamine.
Glycosaminoglycans
Theothercomponent(exceptinthecaseofkeratan sulfate)isauronic acid,eitherLglucuronic acid lf t ) i i id ith L l i id (GlcUA)orits5epimer,Liduronic acid(IdUA).
dGlucuronate
liduronate
Glycosaminoglycans
Thereareatleastsevenglycosaminoglycans: e e a e at east se e g ycosa og yca s 1. Hyaluronic acid, 2. 2 chondroitin sulfate sulfate, 3. keratan sulfatesI 4. keratan lf t II 4 k t sulfatesII 5. heparin, 6. heparan sulfate, 7. anddermatan sulfate.
Glycosaminoglycans (GAGs):
Withtheexceptionofhyaluronic acid,alltheGAGs containsulfategroups,eitherasOestersorasNsulfate (inheparinandheparan sulfate). (i h i dh lf t )
Thefunctionsofglycosaminoglycans (GAGs):
Hyaluronic acidisespeciallyhighinconcentrationin embryonictissuesandisthoughttoplayanimportantrole inpermittingcellmigrationduringmorphogenesisand woundRepair.
Thefunctionsofglycosaminoglycans (GAGs):
Chondroitin sulfatesarelocatedatsitesofcalcificationin endochondral boneandincartilage.
Thefunctionsofglycosaminoglycans (GAGs):
keratan sulfateIand dermatan sulfatearepresentinthe cornea,andplayacriticalroleincornealtransparency. cornea and play a critical role in corneal transparency The presence of dermatan sulfate in the sclera may also Thepresenceofdermatan sulfateinthescleramayalso playaroleinmaintainingtheoverallshapeoftheeye. Keratan sulfateIisalsopresentincartilage.
Thefunctionsofglycosaminoglycans (GAGs):
Heparinisanimportantanticoagulant. ItbindswithfactorsIXandXI,butitsmostimportant interactioniswithplasmaantithrombin. interaction is with plasma antithrombin H Heparincanalsobindspecificallytolipoproteinlipase i l bi d ifi ll li i li presentincapillarywalls,causingareleaseofthisenzyme intothecirculation. into the circulation
Thefunctionsofglycosaminoglycans (GAGs):
Heparans havelesssulfategroupsthanheparins
Heparan sulfate areassociatedwiththeplasma membraneofcells,withtheircoreproteinsspanningthe membrane. Theyactasreceptorsandmayalsoparticipateinthe mediationofcellgrowthandcellcellcommunication.
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