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Abnormal cellular copper metabolism in the blotchy mouse.

B Starcher, J A Madaras, D Fisk

The Journal of Nutrition

|August 1, 1978

Summary

Male mice with the blotchy allele exhibit defective copper metabolism, showing reduced gut absorption and hepatic copper levels. Fibroblast copper accumulation suggests a cellular transport or storage defect.

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Area of Science:

Biochemistry
Genetics
Animal Models

Background:

Copper is an essential trace element vital for various cellular processes.
Defects in copper metabolism can lead to severe health consequences.
The blotchy (Moblo/y) allele in mice is associated with X-linked copper metabolism abnormalities.

Purpose of the Study:

To investigate the specific defects in copper metabolism in male mice with the blotchy (Moblo/y) allele.
To characterize copper absorption, distribution, and cellular handling in these mutant mice.

Main Methods:

Analysis of copper absorption from the gut in mutant and control mice.
Quantification of hepatic copper levels.
Measurement of enzyme activities (ceruloplasmin, cytochrome c oxidase, lysyl oxidase) in tissues and cultured fibroblasts.
Assessment of copper accumulation in cultured fibroblasts and identification of associated proteins.

Main Results:

Mutant mice showed significantly reduced copper absorption (64% of controls) and hepatic copper levels (56% of controls).
Lysyl oxidase activity in cultured fibroblasts was markedly decreased (45% of controls), while ceruloplasmin and heart cytochrome c oxidase activities were normal.
Fibroblasts from mutant mice exhibited a five-fold increase in copper accumulation, associated with a 12,000 molecular weight protein.

Conclusions:

The blotchy allele in mice causes a specific defect in copper metabolism, primarily affecting cellular copper handling and lysyl oxidase activity.
Copper accumulation in fibroblasts suggests a potential defect in copper efflux or storage mechanisms.
Further research is needed to identify the specific protein involved and its role in copper transport or sequestration.

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