A nebulina tamén regula as interaccións actina-[[miosina]] ao inhibir a actividade [[ATPase]] dun modo sensible ao [[calcio]]-[[calmodulina]].<ref name="rootwang">{{cite journal |author=Root & Wang |title=Calmodulin-sensitive interaction of human nebulin fragments with actin and myosin |journal=Biochemistry |volume=33 |issue=42 |pages=12581–91 |year=1994 |pmid=7918483 |doi=10.1021/bi00208a008 |last2=Wang |first2=K }}</ref>
As [[mutación]]s na nebulina causan algúns casos do trastorno [[autosómico recesivo]] [[nemalina miopatía nemalínica]].<ref name="pelin">{{cite journal |author=Pelin |title=Mutations in the nebulin gene associated with autosomal recessive nemaline myopathy |journal=Proc Natl Acad Sci USA |volume=96 |issue=5 |pages=2305–10 |year=1999 |pmid=10051637 |doi=10.1073/pnas.96.5.2305 |last2=Hilpelä |first2=P |last3=Donner |first3=K |last4=Sewry |first4=C |last5=Akkari |first5=PA |last6=Wilton |first6=SD |last7=Wattanasirichaigoon |first7=D |last8=Bang |first8=ML |last9=Centner |first9=T |pmc=26779 |display-authors=1 }}</ref>
Unha segunda isoforma máis pequena da nebulina, denominada [[nebuleta]], exprésase no [[músculo cardíaco]].
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== Estrutura ==
TheA structureestrutura of thedo [[dominio SH3 domain]] ofda nebulinnebulina wasdeterminouse determinedpor byespectroscopçia [[Proteinde nuclearresonancia magneticmagnética resonancenuclear spectroscopy|NMR]]de proteínas.<ref name="pmid9514727"/> TheO dominio SH3 domainda fromnebulina nebulinestá iscomposto composed ofde 60 aminoresiduos acidde residues[[aminoácido]]s, ofdos whichcales o 30 percent% isestá inen theestrutura secundaria en [[betafolla sheetbeta]] secondary structure (7 strandscadeas; 18 residuesresiduos).
== Fenotipo knockout ==
AsA partir ofde 2007, twodesenvolvéronse dous modelos de [[rato knockout|ratos mouseknockout]] modelsda fornebulina nebulinpara haveestudar beenmáis developeda tofondo bettera understandsúa itsfunción ''[[in vivo]]'' function. Bang andet colleaguesal.<ref name="bang">{{cite journal |author=Bang |title=Nebulin-deficient mice exhibit shorter thin filament lengths and reduced contractile function in skeletal muscle |journal=J Cell Biol |volume=173 |issue=6 |pages=905–16 |year=2006 |pmid=16769824 |doi=10.1083/jcb.200603119 |last2=Li |first2=X |last3=Littlefield |first3=R |last4=Bremner |first4=S |last5=Thor |first5=A |last6=Knowlton |first6=KU |last7=Lieber |first7=RL |last8=Chen |first8=J |pmc=2063916 |display-authors=1 }}</ref> demonstrateddemostraron thatque os ratos nebulin-knockout micepara diea postnatallynebulina que morren pouco despois do nacemento, havetiñan reducedreducida thina filamentlonxitude dos filamentos lengthfinos do sarcómero, ando que impairedafectaba á [[musclecontracción contraction|contractile functionmuscular]]. A desorganización e dexeneración Postnataldo [[sarcomeresarcómero]] disorganizationpostnatal andaparecían degenerationrapidamente occurrednestres rapidlyratos, ino theseque mice,indica indicatingque thea nebulinnebulina isé essentialesencial forpara maintainingo themantemento structuralda integrityintegridade ofestrutural das [[myofibrilmiofibrila]]s. Witt ande colleaguescolegas<ref name="witt">{{cite journal |author=Witt |title=Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo |journal=EMBO J |volume=25 |issue=16 |pages=3843–55 |year=2006 |pmid=16902413 |doi=10.1038/sj.emboj.7601242 |last2=Burkart |first2=C |last3=Labeit |first3=D |last4=McNabb |first4=M |last5=Wu |first5=Y |last6=Granzier |first6=H |last7=Labeit |first7=S |pmc=1553189 |display-authors=1 }}</ref> hadobtiveron similarresultados resultssimilares innos theirseus mice,ratos. whichEstes alsoratos diedknockout postnatallypara witha reducednebulina thinestán filamentsendo lengthinvestigados and contractile function. These nebulin-knockout mice are being investigated ascomo [[modelo animal|modelos modelanimais]]sofpara a [[nemalinemiopatía myopathynemalínica]].
