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Diseases of the Nervous System
Published in George Feuer, Felix A. de la Iglesia, Molecular Biochemistry of Human Disease, 2020
George Feuer, Felix A. de la Iglesia
Menkes’ disease is characterized by peculiar hair (kinky-hair syndrome), neurological impairment, and severe mental retardation. The cerebral cortex and white matter are extensively degenerated. Copper levels are low in the liver and brain, and high in other tissues. It is likely that the disease is due to defects in the homeostatic control of copper metabolism. There is a relative deficiency of copper-dependent enzymes related to abnormal and metallothionein gene regulation in response to copper.358 In Menkes’ disease, metallothionein synthesis is defective, which binds copper, and other metallothionein binding metals (mercury, cadmium, zinc) are increased. Abnormalities include increased levels of serum lipids.72
Gastrointestinal system
Published in Aida Lai, Essential Concepts in Anatomy and Pathology for Undergraduate Revision, 2018
Wilson’s disease Autosomal recessiveDisorder of copper metabolismCopper accumulates in liver and brainSymptoms: – Kayser–Fleischer rings in corneaInvestigations: – blood tests (reduced serum copper and caeruloplasmin levels)– urinary copper levels (raised)– liver biopsyManagement: – penicillamine (chelates copper)
Problems on Deficiency and Excess of Minerals In Animal Nutrition
Published in Jul Låg, Geomedicine, 2017
The liver plays a major role in copper metabolism, and secondary copper poisoning may thus occur as a consequence of liver damage. When copper intake exceeds requirements and excretion capacity, accumulation of copper will take place, especially in the liver. The liver, however, can store considerable amounts of copper without adverse effect. Thus, in chronic copper poisoning, there is a latent or symptomless accumulation phase which may last for months. In fact, under field conditions with a moderate excess of copper, such accumulation may be so gradual that an age-related accumulation is seen. This is clearly reflected by the fact that, under such circumstances, chronic copper poisoning only occurs in adult animals, especially the older ones.62
Dexmedetomidine enables copper homeostasis in cerebral ischemia/reperfusion via ferredoxin 1
Published in Annals of Medicine, 2023
Qingduo Guo, Meina Ma, Hong Yu, Yuepeng Han, Dong Zhang
High levels of copper ions can cause damage to vascular endothelial cells and neurons [11,12], and the internal mechanism is generally thought to be that copper ions trigger the accumulation of reactive oxygen species in cells and induce oxidative stress. The important protein copper metabolism domain containing 1 (COMMD1), which is involved in the metabolism of cellular copper homeostasis [13], was also significantly elevated in the brain tissue of aged MCAO rats, and the increase was more noticeable than in young MCAO rats. The increase of COMMD1 leads to the accumulation of copper ions in the cells, causing cell damage and even death [14]. The phenomenon and mechanism of cell damage and death caused by copper ions have been explored for a long time. The destabilization of cellular copper metabolism is a major cause, which manifests as the aggregation of fatty acylated proteins mediated by intracellular ferredoxin 1 (FDX1), loss of iron-sulfur cluster proteins, and aberrant mitochondrial respiration [15]. This type, which leads to proteotoxic stress and ultimately induces cell death, has been highlighted.
Altered levels of GST activity, Vit C, TPX and Cu in individuals with long-term sulfur mustard-induced lung complications
Published in Inhalation Toxicology, 2018
Sussan Kaboudanian Ardestani, Ali Taravati, Zahra Kianmehr, Arash Hajizadeh Dastjerdi, Shahryar Pourfarzam, Mohammad Reza Soroush, Mohammad Mehdi Naghizadeh, Tooba Ghazanfari
Various studies have shown that transition metals including Fe, Cu, Cd and several other metals have the ability to generate ROS (Valko et al., 2005). Copper ions display high affinity for thiol and amino groups occurring in proteins. It is well-evidenced that the Cu toxicity is associated not only with its pro-oxidant properties (catalyze the formation of ROS leading to oxidative stress-related damage) (Gaetke & Chow, 2003) but also with irreversible nonspecific binding to thiol groups occurring in proteins non-related to copper metabolism (Letelier et al., 2005, 2006). This binding may lead to damaging consequences to protein structure and modifying their biological functions. Copper binding to thiol groups of cytochrome P450 monooxygenase and GSH-transferase would decreased enzymatic activities (Letelier et al., 2005).
Investigation of Thiol/Disulfide Homeostasis and Ischemia-Modified Albumin Levels in Children with Wilson Disease
Published in Fetal and Pediatric Pathology, 2022
Ferit Durankuş, Yakup Albayrak, Yavuz Tokgöz, Ömer Faruk Beşer, Ramazan Durankuş, Sebahat Çam, Eda Sünnetçi, Ömer Akarsu, Cemil Nural, Özcan Erel
The free copper that accumulates in the hepatocytes is first neutralized by glutathione (GSH) or metallothionein, and WD is closely associated with oxidative stress (OS) [5]. As active copper accumulates, it triggers OS due to Fenton redox chemistry [6, 7]. An increase in OS has been demonstrated in a human study which included patients ages between 8 and 40 (median 14 years) years old [8], as well as in animal studies [9]. The impairment in copper metabolism causes an increase in circulating copper and subsequent copper accumulation in several types of tissues, including the cornea, lens, brain, kidney, and bone marrow [10]. Free copper can cross the blood-brain barrier, so it impairs astrocytes both directly and by OS [11].