Congenic strains of RCS rats with inherited retinal dystrophy.
نویسندگان
چکیده
Two congenic strains of RCS rats, RCS-p/+ and RCS-c, have been developed that differ from the parental strain at genetic loci affecting pigmentation. Inbred RCS rats are pink-eyed, while RCS-p/+ rats produce segregating litters of pink-eyed (p/p) and black-eyed (p/+) offspring, and RCS-c rats are albinos. All the strains are homozygous for the mutant form of the retinal dystrophy gene. The black eye pigment in RCS-p/+ rats slows the progression of the retinal degeneration by about 10 days in the posterior retina and by about 30-35 days in the peripheral retina in the superior half of the eye. No slowing of the disease occurs in the inferior half of the eye along the vertical meridian. All the strains are similar in body weight and litter size, and show a low incidence of cataract and microphthalmia.
منابع مشابه
Apoptosis leads to photoreceptor degeneration in inherited retinal dystrophy of RCS rats.
PURPOSE To determine the pathogenetic mechanism of photoreceptor cell degeneration in the inherited retinal dystrophy in Royal College of Surgeons (RCS) rats. METHODS The dystrophic retinas of the pink-eyed RCS (RCS-rdy-p) rats were examined for DNA fragmentation by agarose gel electrophoresis of retinal DNA and by TdT-mediated biotin-dUDP nick-end labeling (TUNEL) in paraffin sections. Rats ...
متن کاملLocalization of lysosomal enzymes in retinal pigment epithelium of rats with inherited retinal dystrophy.
Four acid hydrolase activities are demonstrable by light microscopy in pigment epithelial cell lysosomes of rats (Royal College of Surgeons--RCS) with inherited retinal dystrophy and in control (Fischer) rats. The enzymes include acid phosphatase, aryl sulfatase, N-acetyl-beta-glucosaminidase, and esterase activities. No marked differences are observed in distribution or staining intensity of l...
متن کاملAn extracellular retinol-binding glycoprotein in the eyes of mutant rats with retinal dystrophy: development, localization, and biosynthesis
Interstitial retinol-binding protein (IRBP) is a soluble glycoprotein in the interphotoreceptor matrix of bovine, human, monkey, and rat eyes. It may transport retinol between the retinal pigment epithelium and the neural retina. In light-reared Royal College of Surgeons (RCS) and RCS retinal dystrophy gene (rdy)+ rats, the amount of IRBP in the interphotoreceptor matrix increased in correspond...
متن کاملSlit-lamp assessment of onset of cataracts in black-eyed, black-hooded retinal dystrophic rats.
Various types of hereditary retinal degeneration have associated posterior subcapsular cataract (PSC). It has been claimed that in the Royal College of Surgeons (RCS) rat model of hereditary retinal dystrophy, the cataract is manifested unpredictably and does not display Mendelian inheritance. It ws shown previously, however, that 100% of pink-eyed retinal dystrophic RCS rats had an onset of bi...
متن کاملLow expression of alphaA-crystallins and rhodopsin kinase of photoreceptors in retinal dystrophy rat.
PURPOSE The Royal College of Surgeons (RCS) rat has been extensively characterized as a model for inherited retinal dystrophy such as retinitis pigmentosa. In the present study, compositions of retinal proteins were compared between RCS (rdy-/-) and control (rdy+/+) rats during progression of the disease to understand the molecular pathologic course of the retinal degeneration. METHODS Protei...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- The Journal of heredity
دوره 66 4 شماره
صفحات -
تاریخ انتشار 1975