Copper deficiency and neurological disorders in man and animals

Zatta, Paolo; Frank, Adrian

doi:10.1016/j.brainresrev.2006.10.001

Cited by 140 publications

(101 citation statements)

References 136 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…To determine the consequences of DmATP7 silencing on copper absorption, we measured the copper content of newly eclosed flies using an atomic absorption spectrophotometer. Previous studies on Menkes patients and mottled-brindled mutant mice demonstrated that mutations in the ATP7A gene interfere with copper transport to the brain (Madsen and Gitlin, 2007;Niciu et al, 2007;Zatta and Frank, 2007). Here we show that, analogous to Menkes patients and brindled mice, silencing of DmATP7 in the gut interferes with copper delivery to the head and the rest of the body of the adult flies (Fig.…”

Section: Research Articlesupporting

confidence: 62%

A Drosophila model of Menkes disease reveals a role for DmATP7 in copper absorption and neurodevelopment

Bahadorani

Marcon

et al. 2010

Disease Models &Amp; Mechanisms

View full text Add to dashboard Cite

SUMMARY Human Menkes disease is a lethal neurodegenerative disorder of copper metabolism that is caused by mutations in the ATP7A copper-transporting gene. In the present study, we attempted to construct a Drosophila model of Menkes disease by RNA interference (RNAi)-induced silencing of DmATP7, the Drosophila orthologue of mammalian ATP7A, in the digestive tract. Here, we show that a lowered level of DmATP7 mRNA in the digestive tract results in a reduced copper content in the head and the rest of the body of surviving adults, presumably owing to copper entrapment in the gut. Similar to Menkes patients, a majority of flies exhibit an impaired neurological development during metamorphosis and die before eclosion. In addition, we show that survival to the adult stage is highly dependent on the copper content of the food and that overexpression of the copper homeostasis gene, metal-responsive transcription factor-1 (MTF-1), enhances survival to the adulthood stage. Taken together, these results highlight the role of DmATP7-mediated copper uptake in the neurodevelopment of Drosophila melanogaster and provide a framework for the analysis of potential gene interactions influencing Menkes disease.

show abstract

Section: Research Articlesupporting

confidence: 62%

A Drosophila model of Menkes disease reveals a role for DmATP7 in copper absorption and neurodevelopment

Bahadorani

Marcon

et al. 2010

Disease Models &Amp; Mechanisms

View full text Add to dashboard Cite

show abstract

“…[67,68] In Alzheimer's disease, copper binds to the b-peptide, and in Parkinson's to the a-synuclein protein. [69] Copper and zinc are linked to free radical damage implicated in the above neurodegenerative diseases but are also involved in protecting against ROS through the Cu-and Zn-superoxide dismutases.…”

Section: Mt and Oxidative Stressmentioning

confidence: 99%

The Metallothionein/Thionein System: An Oxidoreductive Metabolic Zinc Link

2008

View full text Add to dashboard Cite

Metallothioneins (MTs) were discovered more than 50 years ago and identified as low‐molecular weight, sulfhydryl‐rich proteins that were subsequently found to bind zinc predominantly. The binding of seemingly redox inactive zinc ions allows MT to play a central role in oxidoreductive cellular metabolism, cellular zinc distribution and homeostasis. In this interpretive study, we discuss the interaction of MT with physiologically relevant molecules and its effect on zincthiolate bonds. These interactions are linked to recent progress in the functional role of MT in cellular zinc transport, energy production, and protection of the organism against oxidative stress and neurodegenerative diseases.

show abstract

“…A low copper content in the nervous system leads to a deficiency of COX and a decreased synthesis of phospholipids to the neurons (ZATTA & FRANK, 2007). These are the main mechanisms of peripheral nervous system dysfunction reported in this case.…”

Section: Almeida Et Almentioning

confidence: 56%

Peripheral neuropathy in a copper-deficient goat

et al. 2017

View full text Add to dashboard Cite

This report aimed to describe a case of peripheral neuropathy in a copper-deficient goat, and highlights the clinical, and pathological features of the disease. The goat had low body score, hyporexia, alopecia, achromotrichia, left hindlimb protraction, paralysis with dragging of digit and difficulty to stand up and microcytic normochromic anemia. Copper concentration in serum was markedly lower (2.0µmol L-1) whereas the iron serum content was significantly increased (51.0µmol L-1). The main gross alteration was the reduction of the quadriceps vastus laterallis muscle volume. Histologically, there was atrophy of the quadriceps vastus laterallis muscle and presence of satellite cells, infiltration of lymphocytes, macrophages and replacement of the fibers by connective tissue. In the femoral nerve, there was axonal degeneration with myelin sheath expansion and presence of vacuoles, usually in chains and containing axonal debris or macrophages. Clinical, laboratorial and pathologic findings are consistent with peripheral neuropathy due to a severy copper deficiency.

show abstract

Copper deficiency and neurological disorders in man and animals

Cited by 140 publications

References 136 publications

A Drosophila model of Menkes disease reveals a role for DmATP7 in copper absorption and neurodevelopment

A Drosophila model of Menkes disease reveals a role for DmATP7 in copper absorption and neurodevelopment

The Metallothionein/Thionein System: An Oxidoreductive Metabolic Zinc Link

Peripheral neuropathy in a copper-deficient goat

Contact Info

Product

Resources

About