Versican and Fibrillin-1 Form a Major Hyaluronan-Binding Complex in the Ciliary Body

Ohno-Jinno, Akiko; Isogai, Zenzo; Yoneda, Masahiko; Kasai, Kenji; Miyaishi, Osamu; Inoue, Yoko; Kataoka, Takuya; Zhao, Jing.; Li, Huili; Takeyama, Masayuki; Keene, Douglas R.; Sakai, Lynn Y.; Kimata, Koji; Iwaki, Masaya; Zako, Masaru

doi:10.1167/iovs.07-1488

Cited by 27 publications

(21 citation statements)

References 26 publications

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“…Hirani et al (2007) have shown that LTBP2 speciWcally binds to the amino-terminal region of Wbrillin-1, but not to Wbrillin-2; the major Wbrillin-1 binding site resides close to the carboxyl-terminus of LTBP2. Since LTBP2 interacts and co-localizes with Wbrillin-1 (Hirani et al 2007;Ohno-Jinno et al 2008), it is not surprising to Wnd microspherophakia in some of the MSF patients (Maumenee 1981). As the carboxyl-terminus from amino acids 1816-1821 is lost in the mutant LTBP2 in patients from family 1 (Fig.…”

Section: Resultsmentioning

confidence: 99%

A homozygous mutation in LTBP2 causes isolated microspherophakia

et al. 2010

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Microspherophakia is an autosomal-recessive congenital disorder characterized by small spherical lens. It may be isolated or occur as part of a hereditary systemic disorder, such as Marfan syndrome, autosomal dominant and recessive forms of Weill-Marchesani syndrome, autosomal dominant glaucoma-lens ectopia-microspherophakia-stiffness-shortness syndrome, autosomal dominant microspherophakia with hernia, and microspherophakia-metaphyseal dysplasia. The purpose of this study was to map and identify the gene for isolated microspherophakia in two consanguineous Indian families. Using a whole-genome linkage scan in one family, we identified a likely locus for microspherophakia (MSP1) on chromosome 14q24.1-q32.12 between markers D14S588 and D14S1050 in a physical distance of 22.76 Mb. The maximum multi-point lod score was 2.91 between markers D14S1020 and D14S606. The MSP1 candidate region harbors 110 reference genes. DNA sequence analysis of one of the genes, LTBP2, detected a homozygous duplication (insertion) mutation, c.5446dupC, in the last exon (exon 36) in affected family members. This homozygous mutation is predicted to elongate the LTBP2 protein by replacing the last 6 amino acids with 27 novel amino acids. Microspherophakia in the second family did not map to this locus, suggesting genetic heterogeneity. The present study suggests a role for LTBP2 in the structural stability of ciliary zonules, and growth and development of lens.

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Section: Resultsmentioning

confidence: 99%

A homozygous mutation in LTBP2 causes isolated microspherophakia

et al. 2010

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“…b Versican staining is observed from the pars plana to the peripheral retina, but staining toward the posterior pole is weaker than HA staining. c No versican staining is observed at the posterior pole of the retina Int Ophthalmol ciliary body [14], vitreous [15][16][17], trabeculum [18][19][20], and cornea [21]. In particular, an association between versican expression in the ciliary body and HA production has been described.…”

Section: Discussionmentioning

confidence: 99%

Expression of a hyaluronic acid-binding proteoglycan (versican) in the cynomolgus monkey eye

et al. 2016

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The expression of versican, a hyaluronic acid (HA)-binding protein, during the development and differentiation of the retina has been reported. In this study, we performed histochemical and immunohistological analysis of HA and versican from the ciliary body to the retina in cynomolgus monkey eyes. Paraffin-embedded sections of cynomolgus monkey eyes, including from the ciliary body to the macular region, were prepared. The distribution of versican and HA was examined by histochemical and immunohistochemical methods. The sites of HA expression and versican expression in the eye specimens were similar. Expression of HA and versican was observed in the peripheral retina and ciliary body, but not from the macular region to the mid-periphery of the retina. Versican was strongly expressed in the ciliary body, particularly in the non-pigmented ciliary epithelium. Expression in the retina from the periphery to posterior pole gradually decreased. Versican is expressed from the ciliary body to the peripheral retina, but this expression decreases toward the posterior pole. This suggests a physiological function for versican in the peripheral retina.

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“…Figure 4 shows the proposed structural model of the elastichydrated matrix in the dermis. Furthermore, the fibrillin-versican-hyaluronan network is also observed in the ciliary body (16). …”

Section: Versican Is a Chondroitin Sulfate Proteoglycan And Is Criticmentioning

confidence: 94%

Viscoelastic Properties of the Human Dermis and Other Connective Tissues and Its Relevance to Tissue Aging and Aging–Related Disease

Nemoto¹,

Kubota²,

Murasawa³

et al. 2012

Viscoelasticity - From Theory to Biological Applications

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Versican and Fibrillin-1 Form a Major Hyaluronan-Binding Complex in the Ciliary Body

Cited by 27 publications

References 26 publications

A homozygous mutation in LTBP2 causes isolated microspherophakia

A homozygous mutation in LTBP2 causes isolated microspherophakia

Expression of a hyaluronic acid-binding proteoglycan (versican) in the cynomolgus monkey eye

Viscoelastic Properties of the Human Dermis and Other Connective Tissues and Its Relevance to Tissue Aging and Aging–Related Disease

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