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Expression in mouse kidney of membrane copper transporters Atp7a and Atp7b.
Nephron. 2002; 92(3):629-34.N

Abstract

Copper is essential for activity of many enzymes, but is toxic in excess. Several copper proteins are required for copper homeostasis. ATP7A and ATP7B are genes encoding membrane copper transporters. ATP7A, defective in Menkes disease (MNK), is expressed in many tissues involved primarily in copper uptake from dietary sources. ATP7B, defective in Wilson disease (WND), is essential for copper excretion. Although MNK patients have a copper deficiency in most tissues, copper accumulates in proximal tubules in the kidney. WND patients also have copper accumulation in the proximal tubules. In some WND patients this copper accumulation may result in tubular dysfunction, resulting in the increased excretion of low molecular weight substances (e.g. amino acids and calcium). In mouse, we have demonstrated, by in situ hybridization, the expression pattern in the kidney of mouse orthologues, Atp7a and Atp7b, and have confirmed Atp7b expression by immunohistochemistry. Both Atp7a and Atp7b are expressed in glomeruli; however, Atp7b is also seen in the kidney medulla. This suggests that glomeruli are responsible for regulating copper levels in the filtrate. In WND patients, urinary copper levels are extremely high suggesting Atp7b in the loops of Henle may have a role in copper reabsorption.

Authors+Show Affiliations

University of Alberta, Department of Medical Genetics, Edmonton, Alta., Canada.No affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

12372948

Citation

Moore, Steven D P., and Diane W. Cox. "Expression in Mouse Kidney of Membrane Copper Transporters Atp7a and Atp7b." Nephron, vol. 92, no. 3, 2002, pp. 629-34.
Moore SD, Cox DW. Expression in mouse kidney of membrane copper transporters Atp7a and Atp7b. Nephron. 2002;92(3):629-34.
Moore, S. D., & Cox, D. W. (2002). Expression in mouse kidney of membrane copper transporters Atp7a and Atp7b. Nephron, 92(3), 629-34.
Moore SD, Cox DW. Expression in Mouse Kidney of Membrane Copper Transporters Atp7a and Atp7b. Nephron. 2002;92(3):629-34. PubMed PMID: 12372948.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Expression in mouse kidney of membrane copper transporters Atp7a and Atp7b. AU - Moore,Steven D P, AU - Cox,Diane W, PY - 2002/10/10/pubmed PY - 2003/3/13/medline PY - 2002/10/10/entrez SP - 629 EP - 34 JF - Nephron JO - Nephron VL - 92 IS - 3 N2 - Copper is essential for activity of many enzymes, but is toxic in excess. Several copper proteins are required for copper homeostasis. ATP7A and ATP7B are genes encoding membrane copper transporters. ATP7A, defective in Menkes disease (MNK), is expressed in many tissues involved primarily in copper uptake from dietary sources. ATP7B, defective in Wilson disease (WND), is essential for copper excretion. Although MNK patients have a copper deficiency in most tissues, copper accumulates in proximal tubules in the kidney. WND patients also have copper accumulation in the proximal tubules. In some WND patients this copper accumulation may result in tubular dysfunction, resulting in the increased excretion of low molecular weight substances (e.g. amino acids and calcium). In mouse, we have demonstrated, by in situ hybridization, the expression pattern in the kidney of mouse orthologues, Atp7a and Atp7b, and have confirmed Atp7b expression by immunohistochemistry. Both Atp7a and Atp7b are expressed in glomeruli; however, Atp7b is also seen in the kidney medulla. This suggests that glomeruli are responsible for regulating copper levels in the filtrate. In WND patients, urinary copper levels are extremely high suggesting Atp7b in the loops of Henle may have a role in copper reabsorption. SN - 1660-8151 UR - https://www.unboundmedicine.com/medline/citation/12372948/Expression_in_mouse_kidney_of_membrane_copper_transporters_Atp7a_and_Atp7b_ L2 - https://www.karger.com?DOI=10.1159/000064075 DB - PRIME DP - Unbound Medicine ER -