The distribution of copper in neonatal mottled mutant mice after exposure to copper and penicillamine

Hunt, David M.; Port, Ann E.

doi:10.1016/0024-3205(82)90326-5

Cited by 7 publications

(4 citation statements)

References 11 publications

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“…Although liver copper rose in Cu-treated Mobr'y mice, the levels achieved were well below those of the other mice. This response in Mobr/y mice was noted by others following copper treatment (9,10,14). The results of the cur rent studies with a large dose of copper are similar to those in which a smaller dose (1.2 Â¡ig labeled with 87Cu) was administered subcutaneously (15).…”

Section: Discussionsupporting

confidence: 73%

“…Hunt originally reported no rise in brain copper levels in Mobr/y mice following CuCl2 (9). However, most other reports (10,13), including more recent work from Hunt and Port (14) confirm the observations seen in the current studies, namely that the (10) 562 Â±92.3 (4) 628 Â±213 (6) 522 Â±122 (8) 506 Â±134 (14) 498 Â±122 (11)419Â±48.6 (3) 439 Â±63.7 (8) 536 Â±176 (12) 'Values are means Â±so (number of mice in parentheses) of 12-day-old male C57BL mice. Other details of the protocol can be found in table 1.…”

Section: Discussionmentioning

confidence: 97%

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Repletion of Copper-Deficient Mice and Brindled Mice with Copper or Iron

Prohaska

1984

The Journal of Nutrition

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Studies were conducted in suckling mice to investigate copper-dependent anemia. Brindled (Mobr/y) mice, which have a genetic defect that affects copper metabolism, were compared to their normal brothers (Mo+/y) as well as to anemic suckling mice that were copper-deficient (-Cu) because their dams were consuming a diet low in copper and to a fourth group of control suckling mice (+Cu) from copper-supplemented dams. Mice were given a subcutaneous injection of NaCl, FeCl2 or CuCl2 providing 50 micrograms of Na, Fe or Cu, respectively, when 7 days old and were killed 5 days later. Injection of FeCl2 into -Cu mice elevated liver iron 2.7-fold and raised hemoglobin levels to those observed in the +Cu and Mo+/y mice. Ceruloplasmin activity remained low at 5% of control levels. Injection of CuCl2 into -Cu mice resulted in significant increases in body and brain weight, elevations in serum copper and ceruloplasmin activities and hemoglobin levels. Liver copper rose and iron fell, both to levels observed in +Cu and Mo+/y mice. Brain copper and norepinephrine concentrations rose to control levels. Injection of CuCl2 into Mobr/y mice, although resulting in darker pigmentation and normal serum copper and ceruloplasmin levels, failed to stimulate growth or change brain weight. Furthermore, liver and brain copper levels did not rise to normal levels despite significant improvements. Similarly, brain norepinephrine levels rose, but were still below normal. Brindled mice do not respond to copper therapy to the same degree as do -Cu mice. Iron therapy was successful in reversing the anemia of -Cu mice but was without effect on growth or brain development.

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Section: Discussionsupporting

confidence: 73%

Section: Discussionmentioning

confidence: 97%

Repletion of Copper-Deficient Mice and Brindled Mice with Copper or Iron

Prohaska

1984

The Journal of Nutrition

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“…Elevated copper in the cytosolic fraction was also reported for animal models of Menkes disease (20,21). This may reflect impairment of copper delivery to, and entry into, various organelles due to impaired function of the Menkes protein and induction of MT in response to the accumulation of nonutilizable copper.…”

Section: Discussionmentioning

confidence: 80%

Copper Incorporation into Superoxide Dismutase in Menkes Lymphoblasts

Petrović

Comi

Ettinger

1996

Journal of Biological Chemistry

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“…They usually do not survive for >2 weeks. The symp toms of this disease are considered to arise from altered copper metabolism, as copper accumulates in their intestinal mucosa and kidney and is deficient in various organs, including the brain and liver (Hunt, 1974;Camakaris et al, 1979;Hunt and Port, 1982). Owing to this copper deficiency, several copper-dependent enzymes, including cytochrome c oxidase (COX; EC 1.9.3. l), dopamine-P-hydroxylase (DBH; EC 1.14.17.…”

mentioning

confidence: 99%

Biochemical Study on the Critical Period for Treatment of the Mottled Brindled Mouse

Fujii

Ito

Tsuda

et al. 1990

Journal of Neurochemistry

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Hemizygous mottled brindled mice (Mobr/y mice) were treated by subcutaneous injection of copper and were decapitated on postnatal day 14. Cytochrome c oxidase (COX) activity of the brain mitochondria in the mice given 10 micrograms of copper/g on day 4 or 7 showed significant increases compared with that of untreated Mobr/y animals, and these mice had no neurological symptoms. Mice given 10 micrograms of copper/g on day 12 showed neither increases in COX activity nor clinical improvement. The brain levels of copper, noradrenaline, and dopamine in the mice treated on day 12 were the same as those in animals treated on day 4 or 7. The in vitro activities of dopamine-beta-hydroxylase of the brain were also the same among the treated mice, irrespective of the date of treatment. The results indicate that delays in copper treatment produce irreversible changes in COX activity of the brain and lead to clinical unresponsiveness to treatment.

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The distribution of copper in neonatal mottled mutant mice after exposure to copper and penicillamine

Cited by 7 publications

References 11 publications

Repletion of Copper-Deficient Mice and Brindled Mice with Copper or Iron

Repletion of Copper-Deficient Mice and Brindled Mice with Copper or Iron

Copper Incorporation into Superoxide Dismutase in Menkes Lymphoblasts

Biochemical Study on the Critical Period for Treatment of the Mottled Brindled Mouse

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