منابع مشابه
Assessment of retinal structure and function in Ames waltzer mice.
PURPOSE In humans, mutations in protocadherin 15 are known to result in Usher Syndrome type 1F (USH1F). Patients with USH1F are born with profound hearing loss and have visual problems that develop in late childhood. Based on the phenotypic hearing loss and an associated mutation in protocadherin 15 (Pcdh15), the Ames waltzer mice have been presented as potential models for USH1F. To determine ...
متن کاملDouble homozygous waltzer and Ames waltzer mice provide no evidence of retinal degeneration
PURPOSE To determine whether cadherin 23 and protocadherin 15 can substitute for one another in the maintenance of the retina and other tissues in the mouse. Does homozygosity for both v and av mutant alleles (i.e., a double homozygous mouse) cause retinal degeneration or an obvious retinal histopathology? METHODS We generated mice homozygous for both Cdh23(v-6J) and Pcdh15(av-Jfb) alleles. T...
متن کاملAmes Waltzer deaf mice have reduced electroretinogram amplitudes and complex alternative splicing of Pcdh15 transcripts.
PURPOSE Mutations of PCDH15, the gene encoding protocadherin 15, cause either nonsyndromic deafness DFNB23 or Usher syndrome type 1F (USH1F) in humans and deafness with balance problems in Ames waltzer (av) mice. Persons with USH1 usually begin to exhibit signs of retinitis pigmentosa (RP) in early adolescence, but av mice are reported to have functional retinas. In this study, the auditory, vi...
متن کاملAltered methionine metabolism in long living Ames dwarf mice.
Ames dwarf mice (df/df) are deficient in growth hormone, prolactin, and thyroid-stimulating hormone and live significantly longer than their normal siblings. In the current study, we found that the hormone deficiencies affect methionine metabolism. We previously reported that the dwarf mice exhibit enzyme activities and levels that combat oxidative stress more efficiently than those of normal m...
متن کاملPrimordial follicle activation in the ovary of Ames dwarf mice
BACKGROUND The insulin receptor substrate 1 (IRS1), phosphoinositide 3-kinase (Pi3k), protein kinase B (Akt1), Forkhead Box O3a (FOXO3a) pathway is directly involved in aging and ovarian activation of follicle growth. Therefore, the aim of this work was to measure the expression of genes related to the ovarian pathway for activation of primordial follicles and FOXO3a protein phosphorylation bet...
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ژورنال
عنوان ژورنال: AUDIOLOGY JAPAN
سال: 1971
ISSN: 1883-7301,0303-8106
DOI: 10.4295/audiology.14.347