Cytochrome c oxidase subunit 3 (Mtco3) Recombinant Protein | Mtco3 recombinant protein
Recombinant Mouse Cytochrome c oxidase subunit 3 (Mtco3)
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: Mitochondrial; Membrane protein, integral; Oxidoreductase; Membrane protein, multi-pass
Cellular Component: mitochondrion; membrane; mitochondrial inner membrane; integral to membrane
Molecular Function: cytochrome-c oxidase activity; heme-copper terminal oxidase activity
Biological Process: aerobic electron transport
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Product Notes
The Cytochrome c oxidase subunit 3 (Mtco3) mtco3 (Catalog #AAA1174869) is a Recombinant Protein produced from E Coli or Yeast or Baculovirus or Mammalian Cell and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 1-261. The amino acid sequence is listed below: MTHQTHAYHM VNPSPWPLTG AFSALLLTSG LVMWFHYNSI TLLTLGLLTN ILTMYQWWRD VIREGTYQGH HTPIVQKGLR YGMILFIVSE VFFFAGFFWA FYHSSLVPTH DLGGCWPPTG ISPLNPLEVP LLNTSVLLAS GVSITWAHHS LMEGKRNHMN QALLITIMLG LYFTILQASE YFETSFSISD GIYGSTFFMA TGFHGLHVII GSTFLIVCLL RQLKFHFTSK HHFGFEAAAW YWHFVDVIWL FLYVSIYWWG S. It is sometimes possible for the material contained within the vial of "Cytochrome c oxidase subunit 3 (Mtco3), Recombinant Protein" 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
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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