Wilson's disease and other neurological copper disorders
نویسندگان
چکیده
منابع مشابه
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1 Department of Neuroscience, AFaR-Ospedale “San Giovanni Calibita” Fatebenefratelli, Isola Tiberina, 00186 Rome, Italy 2 Neurodegeneration Laboratory, IRCCS San Raffaele Pisana, 00166 Rome, Italy 3 Department of Neurology, University Hospital Utrecht, 3584 CX Utrecht, The Netherlands 4Departments of Human Genetics and Internal Medicine, University of Michigan Medical School, Ann Arbor, MI 4810...
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Copper is a trace element present in all tissues and is required for cellular respiration, peptide amidation, neurotransmitter biosynthesis, pigment formation, and connective tissue strength. Copper is a cofactor for numerous enzymes and plays an important role in central nervous system development; low concentrations of copper may result in incomplete development, whereas excess copper maybe i...
متن کاملXIAP Is a copper binding protein deregulated in Wilson's disease and other copper toxicosis disorders.
X-linked inhibitor of apoptosis (XIAP), known primarily for its caspase inhibitory properties, has recently been shown to interact with and regulate the levels of COMMD1, a protein associated with a form of canine copper toxicosis. Here, we describe a role for XIAP in copper metabolism. We find that XIAP levels are greatly reduced by intracellular copper accumulation in Wilson's disease and oth...
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ژورنال
عنوان ژورنال: The Lancet Neurology
سال: 2015
ISSN: 1474-4422
DOI: 10.1016/s1474-4422(14)70190-5