نتایج جستجو برای: human copper chaperone

تعداد نتایج: 1728573  

Journal: :The Biochemical journal 2008
Jesse Bertinato Eleonora Swist Louise J Plouffe Stephen P J Brooks Mary R L'abbé

Ctr1 (copper transporter 1) mediates high-affinity copper uptake. Ctr2 (copper transporter 2) shares sequence similarity with Ctr1, yet its function in mammalian cells is poorly understood. In African green monkey kidney COS-7 cells and rat tissues, Ctr2 migrated as a predominant band of approximately 70 kDa and was most abundantly expressed in placenta and heart. A transiently expressed hCtr2-...

Journal: :FEBS letters 2005
Salah E Abdel-Ghany Jason L Burkhead Kathryn A Gogolin Nuria Andrés-Colás Jared R Bodecker Sergi Puig Lola Peñarrubia Marinus Pilon

In plant chloroplasts two superoxide dismutase (SOD) activities occur, FeSOD and Cu/ZnSOD, with reciprocal regulation in response to copper availability. This system presents a unique model to study the regulation of metal-cofactor delivery to an organelle. The Arabidopsis thaliana gene AtCCS encodes a functional homolog to yeast Ccs1p/Lys7p, a copper chaperone for SOD. The AtCCS protein was lo...

Journal: :Neuron 2004
Jian Liu Concepción Lillo P.Andreas Jonsson Christine Vande Velde Christopher M Ward Timothy M Miller Jamuna R Subramaniam Jeffery D Rothstein Stefan Marklund Peter M Andersen Thomas Brännström Ole Gredal Philip C Wong David S Williams Don W Cleveland

One cause of amyotrophic lateral sclerosis (ALS) is mutation in ubiquitously expressed copper/zinc superoxide dismutase (SOD1), but the mechanism of toxicity to motor neurons is unknown. Multiple disease-causing mutants, but not wild-type SOD1, are now demonstrated to be recruited to mitochondria, but only in affected tissues. This is independent of the copper chaperone for SOD1 and dismutase a...

Journal: :Biochemical Society transactions 2007
C Dingwall

The amyloidogenic processing pathway of the APP (amyloid precursor protein) generates Abeta (amyloid beta-peptide), the major constituent in Alzheimer's disease senile plaques. This processing is catalysed by two unusual membrane-localized aspartic proteinases, beta-secretase [BACE1 (beta-site APP-cleaving enzyme 1)] and the gamma-secretase complex. There is a clear link between APP processing ...

2011
Bernd Kastenholz

Commercially available medicinal plant extracts such as Ginkgo biloba leaf extract show no consistent pattern of clinical benefit for people with dementia or cognitive impairment, and have been suggested to be toxic to cells at higher doses. However, medicinal plants may contain other more efficient bioactive molecules apart from the well-known flavonoids and terpenoids. Recombinant therapeutic...

Nerve growth factor (NGF) is a neurotrophic factor that is functional in the survival, maintenance and differentiation of nervous system cells. This protein has three subunits, of which the beta subunit has the main activity. Its structure consists of a cysteine knot motif made up of beta strands linked by disulfide bonds. It can be used as a therapeutic agent in the treatment of many diseases....

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2000
P C Wong D Waggoner J R Subramaniam L Tessarollo T B Bartnikas V C Culotta D L Price J Rothstein J D Gitlin

Recent studies in Saccharomyces cerevisiae suggest that the delivery of copper to Cu/Zn superoxide dismutase (SOD1) is mediated by a cytosolic protein termed the copper chaperone for superoxide dismutase (CCS). To determine the role of CCS in mammalian copper homeostasis, we generated mice with targeted disruption of CCS alleles (CCS(-/-) mice). Although CCS(-/-) mice are viable and possess nor...

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