Rat Cytochrome c oxidase subunit 3 ELISA Kit | Mtco3 elisa kit
Rat Cytochrome c oxidase subunit 3 ELISA Kit
NCBI and Uniprot Product Information
Uniprot Description
COX3: Subunits I, II and III form the functional core of the enzyme complex. Defects in MT-CO3 are a cause of Leber hereditary optic neuropathy (LHON). LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes. Defects in MT-CO3 are a cause of mitochondrial complex IV deficiency (MT-C4D); also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome. Defects in MT-CO3 are associated with recurrent myoglobinuria mitochondrial (RM-MT). Recurrent myoglobinuria is characterized by recurrent attacks of rhabdomyolysis (necrosis or disintegration of skeletal muscle) associated with muscle pain and weakness, and followed by excretion of myoglobin in the urine. Belongs to the cytochrome c oxidase subunit 3 family.
Protein type: Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: -
Cellular Component: mitochondrion
Biological Process: respiratory chain complex IV assembly
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Product Notes
The Rat Mtco3 mtco3 (Catalog #AAA9426373) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9426373 ELISA Kit recognizes Rat Mtco3. It is sometimes possible for the material contained within the vial of "Cytochrome c oxidase subunit 3, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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