نتایج جستجو برای: atp7b

تعداد نتایج: 482  

Journal: :Gastroenterology 2012
Dominik Huster Angelika Kühne Ashima Bhattacharjee Lily Raines Vanessa Jantsch Johannes Noe Wiebke Schirrmeister Ines Sommerer Osama Sabri Frieder Berr Joachim Mössner Bruno Stieger Karel Caca Svetlana Lutsenko

BACKGROUND & AIMS Wilson disease is a severe disorder of copper metabolism caused by mutations in ATP7B, which encodes a copper-transporting adenosine triphosphatase. The disease presents with a variable phenotype that complicates the diagnostic process and treatment. Little is known about the mechanisms that contribute to the different phenotypes of the disease. METHODS We analyzed 28 varian...

Journal: :Oncology reports 2007
Kunio Yoshizawa Shinichi Nozaki Hiroko Kitahara Teruhisa Ohara Koroku Kato Shuichi Kawashiri Etsuhide Yamamoto

Acquired resistance to cisplatin (CDDP) is an issue in cancer chemotherapy. This resistance has been reported to be correlated with the expression of the Cu influx copper transporter 1 (CTR1) and two copper efflux transporters (ATP7A, ATP7B). We investigated the correlation between the expression of these transporters and the sensitivity to CDDP using three pairs of parent cell lines and resist...

2016
Arnab Gupta Michael J. Schell Ashima Bhattacharjee Svetlana Lutsenko Ann L. Hubbard

The cellular machinery responsible for copper-stimulated delivery of the Wilson Disease protein ATP7B to the apical domain of hepatocytes is poorly understood. We demonstrate that myosin Vb regulates the copper-stimulated delivery of ATP7B to the apical domain of polarized hepatic cells, and that disruption of the ATP7B-myosin Vb interaction reduces ATP7B apical surface expression. Myosin Vb ta...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2006
Oleg Dmitriev Ruslan Tsivkovskii Frits Abildgaard Clinton T Morgan John L Markley Svetlana Lutsenko

Wilson disease protein (ATP7B) is a copper-transporting P(1B)-type ATPase that regulates copper homeostasis and biosynthesis of copper-containing enzymes in human tissues. Inactivation of ATP7B or related ATP7A leads to severe neurodegenerative disorders, whereas their overexpression contributes to cancer cell resistance to chemotherapeutics. Copper-transporting ATPases differ from other P-type...

2009
Abdul Khaliq Asifa Majeed

Wilson disease (WND) is an autosomal recessive disorder caused by mutation in ATP7B gene that impairs copper metabolism. ATP7B is involved in the transport of copper into the plasma protein ceruloplasmin and copper excretion out of the liver. Defects in ATP7B lead to excess of copper in various organs primarily in liver. The diagnosis of WND is more complex due to variations in its biochemical ...

Journal: :Journal of hepatology 2016
Jayanta Roy-Chowdhury Michael L Schilsky

Wilson disease (WD) was a once progressive and uniformly fatal inherited disorder of copper metabolism. Medical therapy to arrest progression or prevent complications of WD was developed in the 1950s with the introduction of parenterally administered BAL [1], and over the next two decades by oral therapy with d-penicillamine, trientine, and zinc [2]. Effective therapy for WD requires life-long ...

Journal: :The Biochemical journal 2009
Nataliya V Dolgova Doug Olson Svetlana Lutsenko Oleg Y Dmitriev

Wilson disease ATPase (ATP7B) has been implicated in the resistance of cancer cells to cisplatin. Using a simple in vivo assay in bacterial culture, in the present study we demonstrate that ATP7B can confer resistance to cisplatin by sequestering the drug in its N-terminal metal-binding domain without active drug extrusion from the cell. Expression of a protein fragment containing four N-termin...

E Rezaei , H Dastsooz , H Faraji , J Manoochehri , Kh Sadeghi , M Fardaei , R Masoumi Dehshiri , S Mohammadi , T Moradi ,

Background Wilson disease (WD) is a rare autosomal recessive disorder, which leads to copper metabolism, due to mutations in ATP7B gene. The gene responsible for WD consists of 21 exons that span a genomic region of about 80 kb and encodes a copper transporting P-type ATPase (ATP7B), a protein consisting of 1465 amino acids. Identifying mutation in ATP7B gene is important to find carrier i...

Journal: :Cancer research 2000
M Komatsu T Sumizawa M Mutoh Z S Chen K Terada T Furukawa X L Yang H Gao N Miura T Sugiyama S Akiyama

The accumulation of cisplatin is decreased in many cisplatin-resistant cell lines, and an active efflux pump for cisplatin exists in some of them, but it has not yet been identified. In this study, we transfected the copper-transporting P-type ATPase cDNA (ATP7B) into human epidermoid carcinoma KB-3-1 cells. The transfectant, KB/WD cell line, which overexpressed the P-type ATPase, ATP7B, was re...

Journal: :Biochemical Society transactions 2008
Svetlana Lutsenko

Wilson's disease is a severe human disorder of copper homoeostasis. The disease is associated with various mutations in the ATP7B gene that encodes a copper-transporting ATPase, and a massive accumulation of copper in the liver and several other tissues. The most frequent disease manifestations include a wide spectrum of liver pathologies as well as neurological and psychiatric abnormalities. A...

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