Impact of copper deficiency in humans

Prohaska, Joseph R.

doi:10.1111/nyas.12354

Cited by 70 publications

(45 citation statements)

References 37 publications

(63 reference statements)

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“…There are only about a dozen enzymes in humans that bind copper (31). The vast majority of intracellular copper is bound primarily by two enzymes, cytochrome c oxidase and Cu,Zn SOD.…”

Section: Discussionmentioning

confidence: 99%

Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SODG93A mice co-expressing the Copper-Chaperone-for-SOD

Williams

Trías

Beilby

et al. 2016

Neurobiology of Disease

135

183

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Over-expression of mutant copper, zinc superoxide dismutase (SOD) in mice induces ALS and has become the most widely used model of neurodegeneration. However, no pharmaceutical agent in twenty years has extended lifespan by more than a few weeks. The Copper-Chaperone-for-SOD (CCS) protein completes the maturation of SOD by inserting copper, but paradoxically human CCS causes mice co-expressing mutant SOD to die within two weeks of birth. Hypothesizing that co-expression of CCS created copper deficiency in spinal cord, we treated these pups with the PET-imaging agent CuATSM, which is known to deliver copper into the CNS within minutes. CuATSM prevented the early mortality of CCSxSOD mice, while markedly increasing Cu,Zn SOD protein in their ventral spinal cord. Remarkably, continued treatment with CuATSM extended the survival of these mice by an average of 18 months. When CuATSM treatment was stopped, these mice developed ALS-related symptoms and died within three months. Restoring CuATSM treatment could rescue these mice after they became symptomatic, providing a means to start and stop disease progression. All ALS patients also express human CCS, raising the hope that familial SOD ALS patients could respond to CuATSM treatment similarly to the CCSxSOD mice.

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“…There are only about a dozen enzymes in humans that bind copper (31). The vast majority of intracellular copper is bound primarily by two enzymes, cytochrome c oxidase and Cu,Zn SOD.…”

Section: Discussionmentioning

confidence: 99%

Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SODG93A mice co-expressing the Copper-Chaperone-for-SOD

Williams

Trías

Beilby

et al. 2016

Neurobiology of Disease

135

183

View full text Add to dashboard Cite

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“…Moreover, as FPN has not been shown to export Cu, the decrease in Cu levels may be a secondary effect of lowered intracellular Fe due to increased Fe efflux. Fe-deficiency anaemia has been associated with copper deficiencies, though the mechanism remains unknown 45, 46 . Though future studies are needed to further elucidate FPN’s transport profile in C. elegans , the rescue of the pdr-1 mutant phenotype through FPN overexpression supports our hypothesis that metal dyshomeostasis in the background of pdr-1 loss may be due to alterations in transporter expression.…”

Section: Discussionmentioning

confidence: 99%

Loss of pdr-1/parkin influences Mn homeostasis through altered ferroportin expression in C. elegans

et al. 2015

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Overexposure to the essential metal manganese (Mn) can result in an irreversible condition known as manganism that shares similar pathophysiology with Parkinson's disease (PD), including dopaminergic (DAergic) cell loss that leads to motor and cognitive impairments. However, the mechanisms behind this neurotoxicity and its relationship with PD remain unclear. Many genes confer risk for autosomal recessive, early-onset PD, including the parkin/PARK2 gene that encodes for the E3 ubiquitin ligase Parkin. Using Caenorhabditis elegans (C. elegans) as an invertebrate model that conserves the DAergic system, we previously reported significantly increased Mn accumulation in pdr-1/parkin mutants compared to wildtype (WT) animals. For the current study, we hypothesize that this enhanced accumulation is due to alterations in Mn transport in the pdr-1 mutants. While no change in mRNA expression of the major Mn importer proteins (smf-1-3) was found in pdr-1 mutants, significant downregulation in mRNA levels of the putative Mn exporter ferroportin (fpn-1.1) was observed. Using a strain overexpressing fpn-1.1 in worms lacking pdr-1, we show evidence for attenuation of several endpoints of Mn-induced toxicity, including survival, metal accumulation, mitochondrial copy number and DAergic integrity, compared to pdr-1 mutants alone. These changes suggest a novel role of pdr-1 in modulating Mn export through altered transporter expression, and provides further support of metal dyshomeostasis as a component of Parkinsonism pathophysiology.

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“…Cu is involved in absorption, storage, and metabolism of Fe, as well as cellular metabolism and connective tissue formation (Sun et al, ). It is also important for the catalytic activities of many enzymes, such as cytochrome c oxidase and amine oxidases (Prohaska, ). Se is an essential trace mineral for the basic functions of life and is involved in combating oxidative damage at the cellular level.…”

Section: Resultsmentioning

confidence: 99%

Cultivar selection as a tool for nutritional and functional value enhancement of roasted sweet potato

Hou

et al. 2019

J Food Process Preserv

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In the present study, the nutritional and functional components of fresh and roasted sweet potatoes from 11 cultivars were determined. Results showed that the fresh and roasted sweet potatoes were both rich in protein (3.54–10.34 g/100 g) and dietary fiber (1.81–8.66 g/100 g), with low gross energy (1,576.12–1,638.94 kJ/100 g) and fat (0.75–1.09 g/100 g). After roasting, starch (from 59.46 to 54.43 g/100 g) and protein (from 8.77 to 7.90 g/100 g) contents decreased, while total polyphenol content (from 7.46 to 10.74 mg CHAE/g) and antioxidant activity (from 2.42 to 2.72 μg TE/mg) increased. In addition, most vitamins are preserved after roasting. Gray relational analysis suggested that Yanshu No.25 exhibited the best comprehensive nutritional values (with a weighted gray correlative degree of 0.7787), followed by Longshu No.9 (0.7356). The above results could provide some data support for the research and development, and commercial production of roasted sweet potato with stable high quality. Chemical compounds studied in this article: Chlorogenic acid (PubChem CID:1794427); sulfuric acid (PubChem CID:1118); acetonitrile (PubChem CID:6342); disodium hydrogen phosphate (PubChem CID:24203); acetic acid (PubChem CID:176); sodium hydroxide (PubChem CID:14798); hydrochloric acid (PubChem CID:313); alpha‐D‐glucose (PubChem CID:79025); sodium carbonate (PubChem CID:10340); and ethanol (PubChem CID:702). Practical applications Roasted sweet potato has become a popular product for consumers because it is famous for its excellence in color, aroma, and taste. As roasted sweet potatoes are mainly processed in small workshops, without considering the cultivar issue, the quality of roasted sweet potato products could not be guaranteed, which have caused a large loss of consumers. So far, there is little information on the analysis of different sweet potato cultivars, especially the effects of roasting on the changes in nutritional qualities of different cultivars. The results of this study could provide some data support for the research and development, and commercial production of roasted sweet potato with stable high quality, which could further promote the healthy development of roasted sweet potato industry.

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Impact of copper deficiency in humans

Cited by 70 publications

References 37 publications

Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SODG93A mice co-expressing the Copper-Chaperone-for-SOD

Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SODG93A mice co-expressing the Copper-Chaperone-for-SOD

Loss of pdr-1/parkin influences Mn homeostasis through altered ferroportin expression in C. elegans

Cultivar selection as a tool for nutritional and functional value enhancement of roasted sweet potato

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