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Intracellular localization of the Menkes and Wilson's disease proteins and their role in intracellular copper transport.
Pediatr Int. 1999 Aug; 41(4):436-42.PI

Abstract

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.

Authors+Show Affiliations

Second Department of Pediatrics, Toho University School of Medicine, Tokyo, Japan. mariko-s@remus.dti.ne.jpNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

10453201

Citation

Suzuki, M, and J D. Gitlin. "Intracellular Localization of the Menkes and Wilson's Disease Proteins and Their Role in Intracellular Copper Transport." Pediatrics International : Official Journal of the Japan Pediatric Society, vol. 41, no. 4, 1999, pp. 436-42.
Suzuki M, Gitlin JD. Intracellular localization of the Menkes and Wilson's disease proteins and their role in intracellular copper transport. Pediatr Int. 1999;41(4):436-42.
Suzuki, M., & Gitlin, J. D. (1999). Intracellular localization of the Menkes and Wilson's disease proteins and their role in intracellular copper transport. Pediatrics International : Official Journal of the Japan Pediatric Society, 41(4), 436-42.
Suzuki M, Gitlin JD. Intracellular Localization of the Menkes and Wilson's Disease Proteins and Their Role in Intracellular Copper Transport. Pediatr Int. 1999;41(4):436-42. PubMed PMID: 10453201.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Intracellular localization of the Menkes and Wilson's disease proteins and their role in intracellular copper transport. AU - Suzuki,M, AU - Gitlin,J D, PY - 1999/8/24/pubmed PY - 1999/8/24/medline PY - 1999/8/24/entrez SP - 436 EP - 42 JF - Pediatrics international : official journal of the Japan Pediatric Society JO - Pediatr Int VL - 41 IS - 4 N2 - 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. SN - 1328-8067 UR - https://www.unboundmedicine.com/medline/citation/10453201/Intracellular_localization_of_the_Menkes_and_Wilson's_disease_proteins_and_their_role_in_intracellular_copper_transport_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=1328-8067&date=1999&volume=41&issue=4&spage=436 DB - PRIME DP - Unbound Medicine ER -