-
Albrecht von Graæes Archiv für Ophthalmologie 11/2011; · 2.17 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Alterations in ubiquitin-proteasome system (UPS) have been implicated in the etiology of human cardiovascular diseases. Skp1/Cul1/F-box (SCF) ubiquitin E3 ligase complex plays a pivotal role in ubiquitination of cardiac proteins. However, a specific ubiquitin E3 ligase responsible for the destruction of cardiac transcription factors such as Nkx2-5, Isl1, Mef2C, and Tbx5 remains elusive to date. Here, we show that a novel F-box containing Fbxo25 is cardiac-specific and acts as an ubiquitin E3 ligase for cardiac transcription factors. Fbxo25 expression was nuclei-specific in vitro and cardiomyocytes. Expression level of Fbxo25 was higher in a fetal heart than an adult. Moreover, Fbxo25 expression was increased along with those of cardiac-specific genes during cardiomyocyte development from ESCs. Fbxo25 expression facilitated protein degradation of Nkx2-5, Isl1, Hand1, and Mef2C. Especially, Fbxo25 ubiquitinated Nkx2-5, Isl1, and Hand1. Altogether, Fbxo25 acts as an ubiquitin E3 ligase to target cardiac transcription factors including Nkx2-5, Isl1, and Hand1, indicating that cardiac protein homeostasis through Fbxo25 has a pivotal impact on cardiac development.
Biochemical and Biophysical Research Communications 05/2011; 410(2):183-8. · 2.48 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: We report a case of traumatic optic neuropathy accompanying a grease gun injury to the orbit. A 48-year-old man with a grease gun injury visited our clinic with decreased visual acuity, proptosis and limited extraocular movement (EOM). Orbital CT revealed a crescent mass of fat in the medial intraconal space. The grease was exuded from a lacerated conjunctival wound. The visual evoked potential (VEP) test demonstrated a decreased response in the left eye. Proptosis and EOM were improved after surgical removal of the grease. Systemic high-dose corticosteroid therapy was administered for suspected traumatic optic neuropathy, after which VEP nearly recovered, while visual acuity was slightly improved. A second surgery for traumatic cataract did not further improve visual acuity.
Korean Journal of Ophthalmology 04/2010; 24(2):134-8.
-
[show abstract]
[hide abstract]
ABSTRACT: A newborn girl presented with massive proptosis of the right eye. Physical and radiologic examination disclosed that the primary orbital mass was confined to the site. A diagnosis of malignant rhabdoid tumor was made by histopathologic examination of an incisional biopsy specimen. Exenteration was performed, and the resection margins were free from tumor cells. However, distant metastasis developed in the liver 1 month after surgery. Despite chemotherapy, the patient died 2 months later due to tumor invasion into the central nervous system, which was confirmed by autopsy. To the best of our knowledge, this is the first case of congenital orbital malignant rhabdoid tumor showing systemic metastasis after exenteration, which suggests the need for aggressive systemic treatment rather than exenteration, even in a case of locally confined tumor.
Ophthalmologica 05/2009; 223(4):274-8. · 1.42 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: In a retrospective study, we evaluated the complications in using the motility peg system (nonsleeved and sleeved) for hydroxyapatite orbital implants in an anophthalmic socket.
Drilling for motility peg placement was performed in 265 patients with hydroxyapatite implantation: nonsleeved peg system (n = 191), sleeved peg system (n = 74). A statistical analysis was performed using the chi-square test.
Extrusion rates were significantly lower in the sleeved peg system (10.8%) compared to the nonsleeved peg system (27.2%) (P =.005). The other complications related to motility peg placement were granulation tissue overgrowth (4.2%), hydroxyapatite exposure around peg head (3.0%), and decentered peg (1.9%).
To minimize peg extrusion, the sleeved peg was better than the nonsleeved peg for use in primary motility peg placement.
Japanese Journal of Ophthalmology 46(1):103-7. · 0.92 Impact Factor
