Mouse Anti-MT-CO3 Recombinant Antibody (CBFYM-2724) (CBMAB-M2917-FY)

Name: Mouse Anti-MT-CO3 Recombinant Antibody (CBFYM-2724)
SKU: CBMAB-M2917-FY
Rating: 5 (1 reviews)

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Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

CBFYM-2724

Application

WB, IP

Specificity

Mouse, Rat, Cattle, Human, S. cerevisiae

Antibody Isotype

IgG2a

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.

More Infomation

Target

Full Name

MITOCHONDRIALLY ENCODED CYTOCHROME C OXIDASE III

Introduction

MT-CO3 is a Protein Coding gene. Diseases associated with MT-CO3 include Leber Hereditary Optic Neuropathy and Genetic Recurrent Myoglobinuria. Among its related pathways are Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. and Gene Expression. Gene Ontology annotations related to this gene include cytochrome-c oxidase activity and heme-copper terminal oxidase activity.

Entrez Gene ID

Human	4514
Mouse	17710
Rat	26204

UniProt ID

Human	P00414
Mouse	P00416
Rat	P05505
Cattle	P00415

Alternative Names

Mitochondrially Encoded Cytochrome C Oxidase III; Cytochrome C Oxidase III; MTCO3; COIII; Cytochrome C Oxidase Polypeptide III; Cytochrome C Oxidase Subunit III; EC 1.9.3.1; COXIII; COX3

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.

Biological Process

Aerobic respiration Source: GO_Central
Cellular respiration Source: ComplexPortal
Mitochondrial electron transport, cytochrome c to oxygen Source: GO_Central
Respiratory chain complex IV assembly Source: UniProtKB

Cellular Location

Mitochondrion inner membrane

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.
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.
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.

Topology

Mitochondrial matrix: 1-15
Helical: 16-34
Mitochondrial intermembrane: 35-40
Helical: 41-66
Mitochondrial matrix: 67-72
Helical: 73-105
Mitochondrial intermembrane: 106-128
Helical: 129-152
Mitochondrial matrix: 153-155
Helical: 156-183
Mitochondrial intermembrane: 184-190
Helical: 191-223
Mitochondrial matrix: 224-232
Helical: 233-256
Mitochondrial intermembrane: 257-261

Saleh Jaweesh, M., Hammadeh, M. E., Dahadhah, F. W., Al Smadi, M. A., Al Zoubi, M. S., Alarjah, M. I. A., & Amor, H. (2022). A lack of a definite correlation between male sub-fertility and single nucleotide polymorphisms in sperm mitochondrial genes MT-CO3, MT-ATP6 and MT-ATP8. Molecular Biology Reports, 49(11), 10229-10238.

Xu, M., Kopajtich, R., Elstner, M., Wang, Z., Liu, Z., Wang, J., ... & Fang, F. (2021). Identification of a novel variant in MT-CO3 causing MELAS. Frontiers in genetics, 12, 638749.

Wang, W., Sun, Y., Lin, Y., Xu, X., Zhao, D., Ji, K., ... & Yan, C. (2021). A novel nonsense variant in MT-CO3 causes MELAS syndrome. Neuromuscular Disorders, 31(6), 558-565.

Chen, W., Wang, P., Lu, Y., Jin, T., Lei, X., Liu, M., ... & Fan, S. (2019). Decreased expression of mitochondrial miR-5787 contributes to chemoresistance by reprogramming glucose metabolism and inhibiting MT-CO3 translation. Theranostics, 9(20), 5739.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Isotype control

For research use only. Not intended for any clinical use.

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