Fibrillin: Perbedaan antara revisi

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'''Fibrillin''' adalah sebuah [[glikoprotein]] yang diperlukan untuk membentuk [[serat elastis]] pada [[jaringan ikat]].<ref name="pmid11911778">{{cite journal | author = Kielty CM, Baldock C, Lee D, Rock MJ, Ashworth JL, Shuttleworth CA | title = Fibrillin: from microfibril assembly to biomechanical function | journal = Philos. Trans. R. Soc. Lond., B, Biol. Sci. | volume = 357 | issue = 1418 | pages = 207–17 | year = 2002 | month = February | pmid = 11911778 | pmc = 1692929 | doi = 10.1098/rstb.2001.1029 | url = }}</ref>

 

[[FBN1|Fibrillin-1]] adalah komponen utama [[mikrofibril]] yang membentuk selubung [[elastin]] amorf. [[Mikrofibril]] diyakini terdiri dari [[polimer]] fibrillin ujung-ke-ujung. Sampai saat ini, tiga bentuk fibrillin telah ditemukan. Protein fibrillin-1 ditemukan oleh Engvall pada tahun 1986,<ref name="pmid3536967">{{cite journal | author = Sakai LY, Keene DR, Engvall E | title = Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibrils | journal = J. Cell Biol. | volume = 103 | issue = 6 Pt 1 | pages = 2499–509 | year = 1986 | month = December | pmid = 3536967 | pmc = 2114568 | doi = 10.1083/jcb.103.6.2499| url = | issn = }}</ref> dan mutasi pada gen FBN1 menyebabkan [[sindrom Marfan]].<ref name="pmid20818846">{{cite journal | author = Dietz HC | title = New therapeutic approaches to mendelian disorders | journal = N. Engl. J. Med. | volume = 363 | issue = 9 | pages = 852–63 | year = 2010 | month = August | pmid = 20818846 | doi = 10.1056/NEJMra0907180 | url = | issn = | last2 = Guttmacher | first2 = Alan E. | last3 = Dietz | first3 = Harry C. }}</ref>

 

 

==== Struktur ====

 

Fibrillin-2 ditemukan pada tahun 1994 oleh Zhang<ref>{{cite journal |author=Zhang H, Apfelroth SD, Hu W, ''et al.'' |title=Structure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matrices |journal=J. Cell Biol. |volume=124 |issue=5 |pages=855–63 |year=1994 |pmid=8120105 |doi=10.1083/jcb.124.5.855 |pmc=2119952}}</ref> dan diduga memainkan peran penting dalam elastogenesis awal. Mutasi pada gen fibrillin-2 dikaitkan dengan [[sindrom Beals]].

 

Fibrillin-3 diyakini terpusat di bagian otak.<ref name="pmid14962672">{{cite journal | author = Corson GM, Charbonneau NL, Keene DR, Sakai LY | title = Differential expression of fibrillin-3 adds to microfibril variety in human and avian, but not rodent, connective tissues | journal = Genomics | volume = 83 | issue = 3 | pages = 461–72 | year = 2004 | month = March | pmid = 14962672 | doi = 10.1016/j.ygeno.2003.08.023 | url = }}</ref> Selain otak, fibrillin-3 juga berada di gonad dan ovarium tikus sawah.

 

Fibrillin-4 baru-baru ini ditemukan pada ikan zebra dan memiliki urutan yang mirip seperti fibrillin-2.<ref name="pmid18816837">{{cite journal | author = Gansner JM, Madsen EC, Mecham RP, Gitlin JD | title = Essential role for fibrillin-2 in zebrafish notochord and vascular morphogenesis | journal = Dev. Dyn. | volume = 237 | issue = 10 | pages = 2844–61 | year = 2008 | month = October | pmid = 18816837 | doi = 10.1002/dvdy.21705 | url = | issn = }}</ref>

 

{{Reflist}}

 

* {{MeshName|Fibrillin}}

 

{{Jaringan ikat}}