Immunohistochemical localization of fibrillin in human ocular tissues. Relevance to the Marfan syndrome.
OBJECTIVE
To better understand the ocular manifestations of the Marfan syndrome, we investigated the distribution of fibrillin in normal human ocular tissues. Fibrillin, a microfibrillar glycoprotein component of the extracellular matrix, has been found to be the defective gene product in the Marfan syndrome.


METHODS
Frozen sections from seven pairs of normal eyes were stained with mouse anti-human fibrillin antibodies using the avidin-biotin immunoperoxidase technique.


RESULTS
In the anterior segment, the following exhibited positive staining for fibrillin: the lens capsule and zonules; connective tissues of the iris, ciliary body, ciliary processes, and conjunctiva; and the basement membrane regions of the corneal epithelium and endothelium of Schlemm's canal. Posteriorly, fibrillin localized to the lamina cribrosa, sclera, choroid, and Bruch's membrane.


CONCLUSIONS
Fibrillin is widely distributed in ocular connective tissues. The implications of defects in these tissues and the resultant ocular abnormalities in the Marfan syndrome such as ectopia lentis and glaucoma are discussed.
Wheatley
H M
HM
Johns Hopkins Center for Hereditary Eye Diseases, Baltimore, Md.
Traboulsi
E I
EI
Flowers
B E
BE
Maumenee
I H
IH
Azar
D
D
Pyeritz
R E
RE
Whittum-Hudson
J A
JA
D000368:Aged; D000369:Aged, 80 and over; D000869:Anterior Eye Segment / Q000737:chemistry; D016570:Bruch Membrane / Q000737:chemistry; D002829:Choroid / Q000737:chemistry; D016326:Extracellular Matrix Proteins / Q000032:analysis; D005123:Eye / Q000737:chemistry*; D000071837:Fibrillins; D006801:Humans; D007124:Immunoenzyme Techniques; D008382:Marfan Syndrome / Q000473:pathology*; D008840:Microfilament Proteins / Q000032:analysis*; D008875:Middle Aged; D012590:Sclera / Q000737:chemistry
D016326:Extracellular Matrix Proteins; D000071837:Fibrillins; D008840:Microfilament Proteins