PDF(Pubmed)
Abstract：
UNASSIGNED: Fabry disease is an X-linked lysosomal storage disorder that results in multi-systemic renal, cardiovascular, and neuropathological damage, including in the eyes. We evaluated anterior segment ocular abnormalities based on age, sex (male and female), and genotype (wild-type, knockout [KO] male, heterozygous [HET] female, and KO female) in a rat model of Fabry disease.
UNASSIGNED: The α-Gal A KO and WT rats were divided into young (6-24 weeks), adult (25-60 weeks), and aged (61+ weeks) groups. Intraocular pressure (IOP) was measured. Eyes were clinically scored for corneal and lens opacity as well as evaluated for corneal epithelial integrity and tear break-up time (TBUT). Anterior chamber depth (ACD) and central corneal thickness (CCT) using anterior segment-optical coherence tomography (AS-OCT).
UNASSIGNED: The Fabry rats showed an age-dependent increase in IOP, predominantly in the male genotype. TBUT was decreased in both male and female groups with aging. Epithelial integrity was defective in KO males and HET females with age. However, it was highly compromised in KO females irrespective of age. Corneal and lens opacities were severely affected irrespective of sex or genotype in the aging Fabry rats. AS-OCT quantification of CCT and ACD also demonstrated age-dependent increases but were more pronounced in Fabry versus WT genotypes.
UNASSIGNED: Epithelial integrity, corneal, and lens opacities worsened in Fabry rats, whereas IOP and TBUT changes were age-dependent. Similarly, CCT and ACD were age-related but more pronounced in Fabry rats, providing newer insights into the anterior segment ocular abnormalities with age, sex, and genotype in a rat model of Fabry disease.
UNASSIGNED: The α-Gal A KO and WT rats were divided into young (6-24 weeks), adult (25-60 weeks), and aged (61+ weeks) groups. Intraocular pressure (IOP) was measured. Eyes were clinically scored for corneal and lens opacity as well as evaluated for corneal epithelial integrity and tear break-up time (TBUT). Anterior chamber depth (ACD) and central corneal thickness (CCT) using anterior segment-optical coherence tomography (AS-OCT).
UNASSIGNED: The Fabry rats showed an age-dependent increase in IOP, predominantly in the male genotype. TBUT was decreased in both male and female groups with aging. Epithelial integrity was defective in KO males and HET females with age. However, it was highly compromised in KO females irrespective of age. Corneal and lens opacities were severely affected irrespective of sex or genotype in the aging Fabry rats. AS-OCT quantification of CCT and ACD also demonstrated age-dependent increases but were more pronounced in Fabry versus WT genotypes.
UNASSIGNED: Epithelial integrity, corneal, and lens opacities worsened in Fabry rats, whereas IOP and TBUT changes were age-dependent. Similarly, CCT and ACD were age-related but more pronounced in Fabry rats, providing newer insights into the anterior segment ocular abnormalities with age, sex, and genotype in a rat model of Fabry disease.
摘要：
■法布里病是一种X连锁溶酶体贮积症，可导致多系统肾脏，心血管,和神经病理学损伤，包括眼睛。我们根据年龄评估眼前节眼部异常，性别（男性和女性），和基因型（野生型，击倒[KO]男性，杂合[HET]雌性，和KO雌性）在法布里病的大鼠模型中。
■将α-GalAKO和WT大鼠分为年轻（6-24周），成人（25-60周），和年龄（61周以上）组。测量眼内压(IOP)。对眼睛进行角膜和晶状体混浊的临床评分，并评估角膜上皮完整性和泪液破裂时间（TBUT）。前房深度（ACD）和中央角膜厚度（CCT）使用前段光学相干断层扫描（AS-OCT）。
■法布里大鼠显示出年龄依赖性的IOP升高，主要在男性基因型。随着年龄的增长，男性和女性组的TBUT均降低。随着年龄的增长，KO男性和HET女性的上皮完整性有缺陷。然而,无论年龄大小，KO女性都高度受损。在衰老的法布里大鼠中，无论性别或基因型如何，角膜和晶状体混浊都受到严重影响。CCT和ACD的AS-OCT定量也显示出年龄依赖性增加，但在Fabry基因型与WT基因型中更为明显。
■上皮完整性，角膜,法布里大鼠的晶状体混浊恶化，而IOP和TBUT的变化与年龄有关。同样，CCT和ACD与年龄有关，但在法布里大鼠中更为明显，随着年龄的增长，提供对眼前节眼部异常的新见解，性别,法布里病大鼠模型的基因型。
■将α-GalAKO和WT大鼠分为年轻（6-24周），成人（25-60周），和年龄（61周以上）组。测量眼内压(IOP)。对眼睛进行角膜和晶状体混浊的临床评分，并评估角膜上皮完整性和泪液破裂时间（TBUT）。前房深度（ACD）和中央角膜厚度（CCT）使用前段光学相干断层扫描（AS-OCT）。
■法布里大鼠显示出年龄依赖性的IOP升高，主要在男性基因型。随着年龄的增长，男性和女性组的TBUT均降低。随着年龄的增长，KO男性和HET女性的上皮完整性有缺陷。然而,无论年龄大小，KO女性都高度受损。在衰老的法布里大鼠中，无论性别或基因型如何，角膜和晶状体混浊都受到严重影响。CCT和ACD的AS-OCT定量也显示出年龄依赖性增加，但在Fabry基因型与WT基因型中更为明显。
■上皮完整性，角膜,法布里大鼠的晶状体混浊恶化，而IOP和TBUT的变化与年龄有关。同样，CCT和ACD与年龄有关，但在法布里大鼠中更为明显，随着年龄的增长，提供对眼前节眼部异常的新见解，性别,法布里病大鼠模型的基因型。
