Intracellular localization of the Menkes and Wilson's disease proteins and their role in intracellular copper transport.

Intracellular localization of the Menkes and Wilson's disease proteins and their role in intracellular copper transport. Research Abstract Details 

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Intracellular localization of the Menkes and Wilson's disease proteins and their role in intracellular copper transport. Abstract Text:

Copper is a heavy metal ion essential for the activity of a variety of enzymes in the body. In excess, copper is a very toxic ion and therefore efficient regulation of its metabolism is required. This is dramatically illustrated by the genetic disorders X-linked Menkes disease and autosomal recessive Wilson's disease. In 1993, both the Menkes and Wilson's genes were isolated and it was found that these genes encode homologous cation copper transporting P-type ATPase proteins. The Menkes protein (ATP7A) is expressed in most tissues, except liver. In contrast, the Wilson's protein (ATP7B) is abundantly expressed in liver. Intracellular localization of those proteins was investigated. Both ATP7A and ATP7B are localized in the trans-Golgi network and post-Golgi vesicular compartment (PGVC) in the cell. This intracellular localization was altered by the copper content present in the cell. This result may support the hypothesis that ATP7A and ATP7B are involved in cellular copper transport and those proteins could be suitable models for elucidating intracellular copper metabolism.

Intracellular localization of the Menkes and Wilson's disease proteins and their role in intracellular copper transport. Publishing Authors By Initials

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Intracellular localization of the Menkes and Wilson's disease proteins and their role in intracellular copper transport. Journal Published:

PUBLICATION TYPE: Review

Journal: Pediatrics international : official journal of the

VOLUME: 41

Page Numbers: 436-42

Journal Abbreviation: Pediatr Int

ISSN: 1328-8067

DAY: 21

MONTH: Aug

YEAR: 1999

Intracellular localization of the Menkes and Wilson's disease proteins and their role in intracellular copper transport. Information

Number of References: 26

LANGUAGE: eng

NlmUniqueID: 100886002

Intracellular localization of the Menkes and Wilson's disease proteins and their role in intracellular copper transport. Keywords Mesh Terms:

KEYWORDS: Menkes Kinky Hair Syndrome

MESH TERMS: metabolism

Chemical & Substance for Abstract: Intracellular localization of the Menkes and Wilson's disease proteins and their role in intracellular copper transport. Information

Substance Name: Copper

Registry Number: 7440-50-8

Grant and Affiliation Information for Intracellular localization of the Menkes and Wilson's disease proteins and their role in intracellular copper transport.

AFFILIATION: Second Department of Pediatrics, Toho University School of Medicine, Tokyo, Japan. mariko-s@remus.dti.ne.jp

Country: AUSTRALIA

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MEDLINETA: Pediatr Int

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