What is mitochondrial complex II?
What is mitochondrial complex II?
Mitochondrial complex II (CII), also called succinate dehydrogenase (SDH), is a central purveyor of the reprogramming of metabolic and respiratory adaptation in response to various intrinsic and extrinsic stimuli and abnormalities.
What is autosomal recessive mitochondrial complex II deficiency?
Mitochondrial complex II deficiency is an autosomal recessive multisystemic metabolic disorder with a highly variable phenotype. Some patients have multisystem involvement of the brain, heart, and muscle with onset in infancy, whereas others have only isolated cardiac or muscle involvement.
What is the purpose of complex II?
Complex II is the only membrane-bound component of the Krebs cycle and in addition functions as a member of the electron transport chain in mitochondria and in many bacteria. A recent X-ray structural solution of members of the complex II family of proteins has provided important insights into their function.
What is complex transport electron 2 chain?
Complex II is a parallel electron transport pathway to complex 1, but unlike complex 1, no protons are transported to the intermembrane space in this pathway. Therefore, the pathway through complex II contributes less energy to the overall electron transport chain process.
What is Cox disease?
Cytochrome C Oxidase (COX) deficiency is a very rare inherited metabolic disorder characterized by a deficiency of the enzyme cytochrome C oxidase or Complex IV. Cytochrome C oxidase is an essential enzyme that is active in subcellular structures that help to regulate energy production (mitochondria).
What kind of disease is mitochondrial Complex II deficiency?
Complex II deficiency is a mitochondrial disease. Mitochondria are specialized compartments in cells that create more than 90% of the energy needed by the body.
What is the treatment for mitochondrial Complex III?
Treatment generally focuses on alleviating symptoms and/or slowing down the progression of the disease, and effectiveness can vary among individuals. [3]
What are the different types of mitochondrial disease?
There are many types of mitochondrial disease. Each disorder produces a spectrum of symptoms and abnormalities that can be confusing to both patients and physicians. Ongoing research and clinical trials offer the best hope for quicker diagnosis and more effective treatments.
What happens when mitochondria don’t work correctly?
Mitochondria are specialized compartments in cells that create more than 90% of the energy needed by the body. In mitochondrial diseases, the mitochondria don’t work correctly resulting in less energy in the cell, cell injury and cell death.