Acylphosphate formation by the Menkes copper ATPase
نویسندگان
چکیده
منابع مشابه
A copper treatable Menkes disease mutation associated with defective trafficking of a functional Menkes copper ATPase.
Copper dependency in humans is most dramatically illustrated in Menkes disease, an X linked recessive copper deficiency disorder that is generally lethal in early childhood. 2 Menkes disease is caused by mutations in a transmembrane copper transporting P type ATPase, MNK (or ATP7A), which is expressed in virtually all non-hepatic tissues. Studies using cultured cells suggest that MNK is located...
متن کاملCopper stabilizes the Menkes copper-transporting ATPase (Atp7a) protein expressed in rat intestinal epithelial cells.
Iron deficiency decreases oxygen tension in the intestinal mucosa, leading to stabilization of hypoxia-inducible transcription factor 2α (Hif2α) and subsequent upregulation of genes involved in iron transport [e.g., divalent metal transporter (Dmt1) and ferroportin 1 (Fpn1)]. Iron deprivation also alters copper homeostasis, reflected by copper accumulation in the intestinal epithelium and induc...
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The calcium-dependent acylphosphate formed by the calcium transport ATPase of cardiac sarcoplasmic reticulum and the calcium-, calmodulin-dependent phosphoester(s) of sarcoplasmic reticulum fractions formed by a calcium-, calmodulin-dependent membrane-bound protein kinase can be distinguished by removal of calcium and/or magnesium by EDTA or hydroxylamine treatment of the acid denaturated membr...
متن کاملEssential role for Atox1 in the copper-mediated intracellular trafficking of the Menkes ATPase.
The metallochaperone Atox1 directly interacts with the copper-transporting ATPases and plays a critical role in perinatal copper homeostasis. To determine the cell biological mechanisms of Atox1 function, intracellular copper metabolism, and Menkes ATPase abundance, localization and trafficking were examined in immortalized fibroblast cell lines derived from Atox1(+/+) and Atox1(-/-) embryos. C...
متن کاملRole of the menkes ATPase in the absorption of both copper and iron.
For well over 30 y, it has been known that at the absorptive and metabolic levels, minerals, both macro and trace, interact with one another. With the knowledge gained in the same time span, greater insight has been established as to how the stores of a particular mineral may regulate the absorption of that same mineral. Additionally, we have a greater understanding of how the concentrations of...
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ژورنال
عنوان ژورنال: FEBS Letters
سال: 1997
ISSN: 0014-5793
DOI: 10.1016/s0014-5793(97)00770-9