Warning: Undefined array key "mm" in /www/wwwroot/www.ai-bt.com/si.php on line 10 Deprecated: trim(): Passing null to parameter #1 ($string) of type string is deprecated in /www/wwwroot/www.ai-bt.com/si.php on line 10 Genetic expression of Menkes disease in cultured astrocytes of the macular mouse.

Literature DB >> 1779648

Genetic expression of Menkes disease in cultured astrocytes of the macular mouse.

H Kodama¹, Y Meguro, T Abe, M H Rayner, K T Suzuki, S Kobayashi, M Nishimura.

Abstract

The copper concentration was investigated in the cultured astrocytes from macular mice, an animal model of Menkes disease. An excessive amount of copper was accumulated in the astrocytes as copper-metallothionein. These results show that the underlying genetic defect of the macular mouse is expressed in the astrocytes. A similar situation may exist in Menkes disease and cause a failure of copper transport to neurones.

Entities: Chemical Disease Gene Species

Mesh：

Substances：
Copper
Metallothionein

Year: 1991 PMID： 1779648 DOI： 10.1007/bf01800470

Source DB: PubMed Journal: J Inherit Metab Dis ISSN： 0141-8955 Impact factor: 4.982

23 in total

1. Uptake of peroxidase from the third ventricle by ependymal cells of the median eminence.

Authors: H Kobayashi; M Wada; H Uemura; M Ueck
Journal: Z Zellforsch Mikrosk Anat Date: 1972

2. Letter: Copper and steely hair.

Authors: N Horn; M Mikkelsen; K Heydorn; E Damsgaard; I Tygstrup
Journal: Lancet Date: 1975-05-31 Impact factor: 79.321

3. Copper deficiency in the mitochondria of cultured skin fibroblasts from patients with Menkes syndrome.

Authors: H Kodama; I Okabe; M Yanagisawa; Y Kodama
Journal: J Inherit Metab Dis Date: 1989 Impact factor: 4.982

4. Extra-hepatic storage of copper: a male foetus suspected of Menkes' disease.

Authors: K Keydorn; E Damsgaard; N Horn; M Mikkelsen; I Tygstrup; S Vestemark; J Weber
Journal: Humangenetik Date: 1975-09-10

5. Copper metabolism in mottled mouse mutants: copper concentrations in tissues during development.

Authors: J Camakaris; J R Mann; D M Danks
Journal: Biochem J Date: 1979-06-15 Impact factor: 3.857

6. Copper metabolism in mottled mouse mutants: distribution of 64Cu in brindled (Mobr) mice.

Authors: J R Mann; J Camakaris; D M Danks
Journal: Biochem J Date: 1979-06-15 Impact factor: 3.857

7. Copper metabolism in mottled mouse mutants: copper therapy of brindled (Mobr) mice.

Authors: J R Mann; J Camakaris; D M Danks; E G Walliczek
Journal: Biochem J Date: 1979-06-15 Impact factor: 3.857

8. High-performance liquid chromatography-inductively coupled plasma profiles of cadmium, zinc, sulphur, and other elements in rat liver supernatants after cadmium injection.

Authors: K T Suzuki; H Sunaga; E Kobayashi; N Sugihira
Journal: J Chromatogr Date: 1987-07-29

9. Metal-dependent properties of metallothionein. Replacement in vitro of zinc in zinc-thionein with copper.

Authors: K T Suzuki; T Maitani
Journal: Biochem J Date: 1981-11-01 Impact factor: 3.857

10. Electron microscopic study on brain of macular mutant mouse after copper therapy.

Authors: T Yamano; M Shimada; A Onaga; H Kawasaki; S Iwane; K Ono; M Nishimura
Journal: Acta Neuropathol Date: 1988 Impact factor: 17.088

17 in total

1. Mottled gene expression and copper distribution in the macular mouse, an animal model for Menkes disease.

Authors: Y Murata; H Kodama; Y Mori; M Kobayashi; T Abe
Journal: J Inherit Metab Dis Date: 1998-06 Impact factor: 4.982

2. Sequence of a Menkes-type Cu-transporting ATPase from rat C6 glioma cells: comparison of the rat protein with other mammalian Cu-transporting ATPases.

Authors: Y Qian; E Tiffany-Castiglioni; E D Harris
Journal: Mol Cell Biochem Date: 1998-04 Impact factor: 3.396

3. Disulfiram enhanced delivery of orally administered copper into the central nervous system in Menkes disease mouse model.

Authors: Takao Hoshina; Satoshi Nozaki; Takashi Hamazaki; Satoshi Kudo; Yuka Nakatani; Hiroko Kodama; Haruo Shintaku; Yasuyoshi Watanabe
Journal: J Inherit Metab Dis Date: 2018-08-21 Impact factor: 4.982

4. Effect of medium copper concentration on the growth, uptake and intracellular balance of copper and zinc in Menkes' and normal control cells.

Authors: M H Rayner; K T Suzuki
Journal: Biometals Date: 1994-07 Impact factor: 2.949