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Mouse Anti-COX4I1 Recombinant Antibody (EC127) (V2LY-0624-LY127)


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Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
EC127
Antibody Isotype
IgG
Application
WB, IHC-P, IF

Basic Information

Immunogen
Recombinant Protein.
Host Species
Mouse
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
WB1:1,000-1:3,000
IHC-P1:50-1:300
IF(CC)1:100-1:300

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

Format
Liquid
Buffer
PBS, pH 7.4, 50% glycerol
Preservative
0.02% sodium azide
Concentration
Batch dependent
Purity
>95% by SDS Page
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
COX4I1 (Cytochrome C Oxidase Subunit 4I1) is a Protein Coding gene. Diseases associated with COX4I1 include Calvarial Hyperostosis and Exocrine Pancreatic Insufficiency. Among its related pathways are Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. and Gene Expression. Gene Ontology (GO) annotations related to this gene include cytochrome-c oxidase activity. An important paralog of this gene is COX4I2.
Entrez Gene ID
Human1327
Mouse12857
Rat29445
UniProt ID
HumanP13073
MouseP19783
RatP10888
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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