MT-CO1
GeneName
MT-CO1
Summary
MT-CO1, also known as COI or cytochrome c oxidase subunit 1, is a 57 kDa protein that is an essential component of the mitochondrial inner membrane, specifically part of the respiratory chain complex IV. It plays a crucial role in aerobic respiration and cellular respiration by facilitating the transfer of electrons from cytochrome c to oxygen, thereby contributing to the mitochondrial electron transport chain. MT-CO1 is involved in various biological processes, including cerebellum development and responses to copper ions, electrical stimuli, hypoxia, and oxidative stress.
Importance
MT-CO1 is relevant to: - Mitochondrial function and energy production, as it is integral to the respiratory electron transport chain - Disorders related to mitochondrial dysfunction, which can lead to a range of diseases including neurodegenerative disorders and metabolic syndromes - Research on hypoxia and oxidative stress responses, providing insights into cellular adaptation mechanisms - Studies on copper ion homeostasis, given its response to copper levels in the cellular environment
Top Products
For researchers investigating MT-CO1, we highly recommend the top-selling recombinant antibody, Anti-MTCO1 antibody [1D6E1A8] (ab14705). This well-cited product has garnered 591 citations, reflecting its reliability and trust within the scientific community. It has been validated for use in several applications, including Western blotting (WB), immunohistochemistry (IHC), immunocytochemistry (ICC), and flow cytometry (FC). The recombinant nature of this antibody ensures batch-to-batch consistency, making it an excellent choice for your research needs. The MitoBiogenesis™ In-Cell ELISA Kit (Fluorescent) for MT-CO1 (ab140359), supported by 2 citations, offers a reliable method for researchers looking to assess mitochondrial biogenesis in their samples.
Abcam Product Citation Summary
The data indicates that the Abcam antibody ab14705 is widely used for detecting MT-CO1 in various species, particularly in mouse and human models. The studies focus on diverse contexts such as mitochondrial function, neurodegeneration, and the effects of chemotherapy on mitochondrial dynamics. The applications primarily involve Western blotting and immunohistochemistry, highlighting the importance of MT-CO1 in understanding mitochondrial-related diseases and conditions.
Abcam Product Citation Table
Function
Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Electrons originating from reduced cytochrome c in the intermembrane space (IMS) are transferred via the dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 to the active site in subunit 1, a binuclear center (BNC) formed by heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 water molecules using 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix.
Involvement in disease
Leber hereditary optic neuropathy
LHON
A maternally inherited form of Leber hereditary optic neuropathy, a mitochondrial disease resulting in bilateral painless loss of central vision due to selective degeneration of the retinal ganglion cells and their axons. The disorder shows incomplete penetrance and male predominance. Cardiac conduction defects and neurological defects have also been described in some LHON patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.
None
The disease is caused by variants affecting the gene represented in this entry.
MT-CO1 may play a role in the pathogenesis of acquired idiopathic sideroblastic anemia, a disease characterized by inadequate formation of heme and excessive accumulation of iron in mitochondria. Mitochondrial iron overload may be attributable to mutations of mitochondrial DNA because these can cause respiratory chain dysfunction, thereby impairing reduction of ferric iron to ferrous iron. The reduced form of iron is essential to the last step of mitochondrial heme biosynthesis.
Mitochondrial complex IV deficiency
MT-C4D
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, exercise intolerance, developmental delay, delayed motor development and intellectual disability. Some affected individuals manifest a fatal hypertrophic cardiomyopathy resulting in neonatal death. A subset of patients manifest Leigh syndrome.
None
The disease is caused by variants affecting the gene represented in this entry.
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.
None
The gene represented in this entry may be involved in disease pathogenesis.
Deafness, sensorineural, mitochondrial
DFNM
A form of non-syndromic deafness with maternal inheritance. Affected individuals manifest progressive, postlingual, sensorineural hearing loss involving high frequencies.
None
The disease is caused by variants affecting the gene represented in this entry.
Colorectal cancer
CRC
A complex disease characterized by malignant lesions arising from the inner wall of the large intestine (the colon) and the rectum. Genetic alterations are often associated with progression from premalignant lesion (adenoma) to invasive adenocarcinoma. Risk factors for cancer of the colon and rectum include colon polyps, long-standing ulcerative colitis, and genetic family history.
None
The gene represented in this entry may be involved in disease pathogenesis.
Pathway
Energy metabolism; oxidative phosphorylation.
Sequence Similarities
Belongs to the heme-copper respiratory oxidase family.
Cellular localization
- Mitochondrion inner membrane
- Multi-pass membrane protein
Alternative names
COI, COXI, MTCO1, MT-CO1, Cytochrome c oxidase subunit 1, Cytochrome c oxidase polypeptide I