MT-CO2

MT-CO2 (Mitochondrially Encoded Cytochrome C Oxidase II) is a Protein Coding gene. Diseases associated with MT-CO2 include Mitochondrial Complex Iv Deficiency and Familial Colorectal Cancer. Among its related pathways are Development EGFR signaling via small GTPases and Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. Gene Ontology (GO) annotations related to this gene include oxidoreductase activity and cytochrome-c oxidase activity.

Mitochondrially Encoded Cytochrome C Oxidase II

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.

ATP synthesis coupled electron transport Source: GO_Central
Cellular respiration Source: ComplexPortal
Lactation Source: Ensembl
Mitochondrial electron transport, cytochrome c to oxygen Source: FlyBase
Positive regulation of ATP biosynthetic process Source: Ensembl
Positive regulation of hydrogen peroxide biosynthetic process Source: Ensembl
Positive regulation of necrotic cell death Source: Ensembl
Response to cold Source: Ensembl

Mitochondrion inner membrane

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 mental retardation. Some affected individuals manifest a fatal hypertrophic cardiomyopathy resulting in neonatal death. A subset of patients manifest Leigh syndrome.

Mitochondrial intermembrane: 1-14
Helical: 15-45
Mitochondrial matrix: 46-59
Helical: 60-87
Mitochondrial intermembrane: 88-227