Wilson's disease: Food therapy out of trace elements

Article Gastroenterology & Hepatology

ATP7B gene therapy of autologous reprogrammed hepatocytes alleviates copper accumulation in a mouse model of Wilson's disease

Hongxia Cai et al.

Summary: This study successfully established a system of autologous reprogrammed Wilson's disease hepatocytes and demonstrated the therapeutic efficacy of ATP7B gene restoration in a mouse model.

HEPATOLOGY (2022)

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Article Neurosciences

Increased Magnetic Susceptibility in the Deep Gray Matter Nuclei of Wilson's Disease: Have We Been Ignoring Atrophy?

Xiao-Zhong Jing et al.

Summary: This study quantitatively assessed the changes of metal concentration and total metal content in the deep gray matter nuclei of patients with Wilson's disease (WD) using quantitative susceptibility mapping. The results showed that mean bulk susceptibility and total susceptibility were higher in WD patients compared to healthy controls. Mean bulk susceptibility of putamen demonstrated the best diagnostic capability for patients with neurological WD, while total susceptibility of pontine tegmentum was most significant for the diagnosis of patients with hepatic WD.

FRONTIERS IN NEUROSCIENCE (2022)

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Article Cell Biology

Copper deficiency affects the developmental competence of porcine oocytes matured in vitro

Hyerin Choi et al.

Summary: This study elucidated the copper transport system in porcine follicular cells and investigated the effect of copper chelation on oocyte maturation and subsequent embryonic development. The results showed that copper chelation negatively affected oocyte maturation, but this effect was alleviated when copper was added. Both copper supplementation and chelation increased reactive oxygen species levels in porcine oocytes. In addition, the expression of MAPK3 in cumulus cells significantly increased under copper chelation conditions. Furthermore, copper chelation led to decreased blastocyst formation rates in parthenogenetically activated embryos.

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY (2022)

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Article Cell Biology

Copper delivery to an endospore coat protein of Bacillus subtilis

Jaeick Lee et al.

Summary: A family of cytosolic copper storage proteins (Csps), specifically Csp3s, bind Cu(I) via their Cys-lined four-helix bundles. The presence of Csp3s in bacteria challenges the belief that bacteria do not maintain intracellular Cu pools like eukaryotes due to its potential toxicity. This study investigates the role of Csp3s in Bacillus subtilis sporulation and suggests that Csp3s may acquire Cu(I) in the cytosol for a target enzyme, BsCotA.

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY (2022)

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Review Environmental Sciences