Abstract：
PURPOSE:To investigate the relationship between ornithine-delta-aminotransferase (OAT) deficiency and ornithine accumulation and the specific degeneration of retinal pigment epithelial (RPE) cells in gyrate atrophy. METHODS:Human RPE cells, human hepatoma cells, and human fibroblast cells were treated with 5-fluoromethylornithine (5-FMOrn), a specific irreversible inhibitor of OAT. Ornithine cytotoxicity was determined by using a [3H]thymidine incorporation assay and immunohistochemical staining for cytokeratin. The effects of various metabolites of ornithine and arginine, such as creatine, creatine phosphate, I-delta 1-pyrroline-5-carboxylic acid (L-P5C), and proline, which may be deficient in gyrate atrophy on RPE cell damage by ornithine, were determined by the same procedures. RESULTS:When the human RPE cells, HepG2 hepatoma cells, and WI-38 fibroblast cells were treated with 0.5 mM 5-FMOrn for 30 minutes, which inactivated OAT, ornithine exhibited severe time- and dose-dependent inhibition of DNA synthesis in the human RPE cells but not in the HepG2 hepatoma cells or WI-38 fibroblast cells. The inhibition of DNA synthesis was accompanied by drastic changes in morphologic appearance, disorganization of the cytoskeleton, and cell death. Ornithine or 5-FMOrn alone did not exhibit such cytotoxicity to the RPE cells. Proline prevented the cytotoxicity of ornithine. CONCLUSIONS:These findings suggest that an elevated level of ornithine combined with an increased sensitivity to ornithine as a result of OAT deficiency may be crucial to the specific RPE degeneration in gyrate atrophy. They suggest also that abnormalities of proline metabolism may be involved in the progress of gyrate atrophy.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Ueda M,Masu Y,Ando A,Maeda H,Del Monte MA,Uyama M,Ito Ssubject
Has Abstractpub_date
1998-04-01 00:00:00pages
820-7issue
5eissn
0146-0404issn
1552-5783journal_volume
39pub_type
杂志文章abstract：PURPOSE:Currently, the test for useful field of view (UFOV) typically relies on subjective feedback for detecting peripheral targets while attending to a central target. Steady-state visual evoked potential (SSVEP) recorded from the occipital region of the brain has been frequently used as an objective alternative to r...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.15-18439
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journal_title：Investigative ophthalmology & visual science
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journal_title：Investigative ophthalmology & visual science
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journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.12-10526
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journal_title：Investigative ophthalmology & visual science
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更新日期：1990-08-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
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doi：
更新日期：1995-04-01 00:00:00
abstract：PURPOSE:To compare directly the robustness of standard automated perimetry (SAP), short-wavelength automated perimetry (SWAP), frequency-doubling perimetry (FDP), and grating-resolution perimetry (GRP) stimuli to different degrees of intraocular stray light induced by commercially available opacity-containing filters. ...
journal_title：Investigative ophthalmology & visual science
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doi：10.1167/iovs.08-2934
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journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1987-10-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
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journal_title：Investigative ophthalmology & visual science
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journal_title：Investigative ophthalmology & visual science
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journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1999-10-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.03-0625
更新日期：2004-03-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
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更新日期：2019-09-03 00:00:00
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journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
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pub_type： 杂志文章
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更新日期：2019-11-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
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doi：10.1167/iovs.07-1234
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journal_title：Investigative ophthalmology & visual science
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journal_title：Investigative ophthalmology & visual science
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journal_title：Investigative ophthalmology & visual science
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doi：
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journal_title：Investigative ophthalmology & visual science
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journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1981-06-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1997-11-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
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doi：
更新日期：2001-02-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
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更新日期：2019-05-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.03-0097
更新日期：2003-08-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.04-1031
更新日期：2005-02-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
pub_type： 临床试验,杂志文章
doi：10.1167/iovs.03-0659
更新日期：2004-01-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1982-02-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.15-16730
更新日期：2015-06-01 00:00:00
