Heterozygous females carrying the brindled allele (Atp7aMo-br) are very similar to mottled heterozygous females (Atp7aMo/+) in appearance but have normal viability. They have curly vibrissae, but the coat is not noticeably waved. Hemizygous males are almost devoid of pigment except in the eyes and ears. The vibrissae are strongly curled, and the coat is wavy. Males usually die by two weeks of age, but a few have lived and been fertile. They have a behavioral abnormality consisting of a slight tremor, uncoordinated gait, and clasping of the hindfeet when held up by the tail. Histological examination of the brain of brindled males shows widespread neuronal degeneration in the cerebral cortex and thalamic nuclei and scattered degeneration in the cerebellum. Heterozygous females have been shown to have neurochemical abnormalities as well. In contrast to mice bearing other Atp7a alleles, brindled mice have no aortic lesions and no defect in crossli...
Heterozygous females carrying the brindled allele (Atp7aMo-br) are very similar to mottled heterozygous females (Atp7aMo/+) in appearance but have normal viability. They have curly vibrissae, but the coat is not noticeably waved. Hemizygous males are almost devoid of pigment except in the eyes and ears. The vibrissae are strongly curled, and the coat is wavy. Males usually die by two weeks of age, but a few have lived and been fertile. They have a behavioral abnormality consisting of a slight tremor, uncoordinated gait, and clasping of the hindfeet when held up by the tail. Histological examination of the brain of brindled males shows widespread neuronal degeneration in the cerebral cortex and thalamic nuclei and scattered degeneration in the cerebellum. Heterozygous females have been shown to have neurochemical abnormalities as well. In contrast to mice bearing other Atp7a alleles, brindled mice have no aortic lesions and no defect in crosslinking of collagen and elastin. Brindled males have defective placental transport and defective intestinal absorption of copper. Parenteral injection of copper at 7 to 10 days of age prevents tremor and early death, allows normal pigmentation, improves growth, produces normal concentrations of copper in organs previously deficient (except liver), producesnormal activity of some copper-dependent enzymes, and prevents neuronal degeneration.
Brindled (Atp7aMo-br) arose spontaneously in the C57BL strain about 1953 (Fraser, A.S. et al. 1953. J Genetics 51:217-221). It was imported into the Jackson Laboratory on the C57BL/6J background in 1995 from Christine Peterson at The Andrus Gerontology Center of the University of Southern California. Female heterozygotes were bred to C57BL/6J and their female heterozygous offspring were again backcrossed to C57BL/6J before hysterectomy rederivation. This strain is maintained by mating a heterozygous brindled female to a C57BL/6J male each generation. In 2000 this strain reached generation +N11 and in 2006 it reached generation +N25.
As hemizygous (Atp7aMo-br/Y) males and homozygous (Atp7aMo-br/Atp7aMo-br)females do not survive to breeding age, this strain must be maintained by breeding heterozygous (Atp7aMo-br/+) females to wild-type males.
The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided,
their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of
each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders
are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in
order to provide the minimum number of animals, animals will ship within 25 weeks.
The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.
Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation.
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