Importance of Copper in ALS and and Huntington’s Disease

Copper is an important cofactor for the functioning of various cuproenzymes that are required for proper brain development and functioning. For example, in superoxide dismutase-1 (SOD1), the binding of copper is necessary to break superoxide radicals that exhibit neurotoxicity. Many cuproenzymes have chaperones, such as copper chaperone for SOD1 (CCS), to facilitate binding of the … Read more

Use of Chelators to Decrease Lewy Body Aggregation in Parkinson’s Disease

For an introduction to Parkinson’s Disease (PD) and the role of metals, see “Atox1-Cu-𝛼Syn Complex Providing Answers about Possible Mechanisms of Aggregation of Lewy Bodies in Parkinson’s Disease” Researchers at Huazhong University of Science and Technology wanted to see if treating C. Elegans with chelating agents would decrease the overproduction and aggregation of 𝛼Syn. Knowing … Read more

Atox1-Cu-𝛼Syn Complex Providing Answers about Possible Mechanisms of Aggregation of Lewy Bodies in Parkinson’s Disease

Parkinson’s Disease (PD) is the second most common neurodegenerative disease after Alzheimer’s disease, affecting about 1-2 people 1000, including about 1% of people over 60. There are genetic markers in 5-10% of patients, but there is no direct connection as many patients develop symptoms without these markers. Symptoms of PD include difficulty walking and talking, … Read more

Metal Complexes Decrease Amyloid-ꞵ Formation and Increase the Concentration of Intracellular Copper and Zinc

Alzheimer’s disease (AD) is the most common neurodegenerative disease, affecting 5.8 million people in the United States, including about ½ of nursing home and hospice patients. AD causes cognitive decline, resulting in extreme memory loss, often to the point where patients may not be able to recognize their own children or spouse. Most patients are … Read more

Discovery of an Atomic Structure of Bacterial Cu+-Transport Protein Reveals Insight About Menkes/Wilson Disease

Copper is an essential metal that we get from daily intake of foods and water. Without it, certain copper-dependent enzymes (“cuproenzymes”) cease to function which has negative consequences in energy production, brain and muscle development, and other biochemical/physiological processes in our bodies [1]. Menkes and Wilson are genetic conditions associated with deficient and toxic levels … Read more

Copper dishomeostasis causes a misbalance in Catecholamine levels

Copper serves as a signaling and regulatory molecule as well as a cofactor of important enzymes in the brain, and is thus required for proper brain development and functioning. Irregularities in copper metabolism can lead to several disorders, including Menkes disease and Wilson’s disease. Menkes disease is the result of genetic mutations altering ATP7A, a … Read more

Using CRISPR/Cas9 to correct a Wilson’s Disease Mutation in Human Cells

Wilson’s Disease (WD) is a liver disease caused by mutations in a protein responsible for copper excretion. That protein, ATPase7B (ATP7B), is a pump that helps move copper in liver cells, and mutations in it cause it to lose its ability to function. The impaired excretion of copper from the liver leads to the accumulation … Read more

Chromium Allergy and its Activation of the Innate Immune Response

Chromium allergy is a type of allergic contact dermatitis characterized by rashes, inflammation, and scaling. Our innate immune response drives this inflammatory reaction when the surface of our skin comes in contact with chromium (hence, the term, “allergic contact dermatitis.”) The primary cause of chromium allergy is constant exposure to chromium-containing chemicals or compounds. The … Read more

Structural and biochemical analysis of the metallo-β-lactamase L1 from emerging pathogen Stenotrophomonas maltophilia revealed the subtle but distinct di-metal scaffold for catalytic activity

The emergence of antibiotic resistance is a major challenge in the clinical setting. Stenotrophomonas maltophilia is an emerging gram-negative bacterium that produces Metallo-b-lactamase (MBL) L1. Metallo-beta-Lactamases (MBLs) is a class B β-lactamases that hydrolyze β-lactam antibiotics. The β-lactam antibiotics, which include antibiotics such as penicillin, inhibit bacterial transpeptidases involved in cell-wall biosynthesis. Bacteria produce MBLs … Read more

Copper supplementation restores cytochrome c oxidase assembly defect in amitochondrial disease model of COA6 deficiency

In general, mitochondrial myopathies are neuromuscular disorders caused by dysfunctional mitochondria. When mitochondria function incorrectly, ATP is not produced, and the cell suffers. With enough cells suffering, an organism can have widespread problems, resulting in systemic failures. Unfortunately, mitochondrial diseases are very promiscuous, and individual mutations in many different proteins can cause each classified disease. … Read more