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Mouse Anti-COX4I1 Recombinant Antibody (CBFYC-2114) (CBMAB-C2181-FY)


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Summary

Host Animal
Mouse
Specificity
Cattle, Human
Clone
CBFYC-2114
Antibody Isotype
IgG2a, k
Application
IF, IHC, WB

Basic Information

Immunogen
Bovine Complex IV subunit IV
Specificity
Cattle, Human
Antibody Isotype
IgG2a, k
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Concentration
1 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Cytochrome C Oxidase Subunit 4I1
Introduction
Cytochrome c oxidase (COX) is the terminal enzyme of the mitochondrial respiratory chain. It is a multi-subunit enzyme complex that couples the transfer of electrons from cytochrome c to molecular oxygen and contributes to a proton electrochemical gradient across the inner mitochondrial membrane. The complex consists of 13 mitochondrial-and nuclear-encoded subunits. The mitochondrially-encoded subunits perform the electron transfer and proton pumping activities. The functions of the nuclear-encoded subunits are unknown but they may play a role in the regulation and assembly of the complex. This gene encodes the nuclear-encoded subunit IV isoform 1 of the human mitochondrial respiratory chain enzyme. It is located at the 3' of the NOC4 (neighbor of COX4) gene in a head-to-head orientation, and shares a promoter with it. Pseudogenes related to this gene are located on chromosomes 13 and 14. Alternative splicing results in multiple transcript variants encoding different isoforms.
Entrez Gene ID
Human1327
Cattle281090
UniProt ID
HumanP13073
CattleP00423
Alternative Names
Cytochrome C Oxidase Subunit 4I1; Cytochrome C Oxidase Subunit IV Isoform 1; Cytochrome C Oxidase Polypeptide IV; Cytochrome C Oxidase Subunit IV; COX IV-1; COX4;
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 subunbit 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.
Biological Process
Generation of precursor metabolites and energy Source: ProtInc
Mitochondrial electron transport, cytochrome c to oxygen Source: GO_Central
Response to nutrient Source: Ensembl
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Mitochondrial complex IV deficiency, nuclear type 16 (MC4DN16):
An autosomal recessive mitochondrial disorder with onset in infancy and variable manifestations. MC4DN16 features include feeding difficulties, poor overall growth, short stature, microcephaly, developmental regression, severe hypotonia, and seizures. Cerebral and cerebellar atrophy, and abnormal lesions in the basal ganglia can be observed on brain imaging. Patient tissues show decreased levels and activity of mitochondrial respiratory complex IV.
Topology
Mitochondrial matrix: 23-98
Helical: 99-124
Mitochondrial intermembrane: 125-169
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For research use only. Not intended for any clinical use.

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