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Mouse Anti-MT-CYB Recombinant Antibody (54.1) (CBMAB-G1148-LY)

This product is antibody recognizes MT-CYB. The antibody 54.1 immunoassay techniques such as: WB.
See all MT-CYB antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse
Clone
54.1
Antibody Isotype
IgG1, κ
Application
WB

Basic Information

Immunogen
Partially purified human neutrophil flavocytochrome b
Specificity
Human, Mouse
Antibody Isotype
IgG1, κ
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
Preservative
0.09% sodium azide
Concentration
0.5 mg/mL
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Mitochondrially Encoded Cytochrome B
Introduction
MT-CYB (Mitochondrially Encoded Cytochrome B) is a Protein Coding gene. Diseases associated with MT-CYB include Cardiomyopathy, Infantile Histiocytoid and Mitochondrial Encephalomyopathy.
Among its related pathways are Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. and Metabolism.
Gene Ontology (GO) annotations related to this gene include oxidoreductase activity and ubiquinol-cytochrome-c reductase activity.
Entrez Gene ID
Human4519
UniProt ID
HumanP00156
MouseP00158
Alternative Names
Mitochondrially Encoded Cytochrome B; Cytochrome B; MTCYB; CYTB; Ubiquinol-Cytochrome-C Reductase Complex Cytochrome B Subunit; Cytochrome B-C1 Complex Subunit 3; Complex III Subunit III; Complex III Subunit 3; Cob;
Function
Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex) that is part of the mitochondrial respiratory chain. The b-c1 complex mediates electron transfer from ubiquinol to cytochrome c. Contributes to the generation of a proton gradient across the mitochondrial membrane that is then used for ATP synthesis.
Biological Process
Cellular respiration Source: ComplexPortal
Mitochondrial electron transport, ubiquinol to cytochrome c Source: UniProtKB
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Defects in MT-CYB are a rare cause of mitochondrial dysfunction underlying different myopathies. They include mitochondrial encephalomyopathy, hypertrophic cardiomyopathy (HCM), and sporadic mitochondrial myopathy (MM). In mitochondrial myopathy, exercise intolerance is the predominant symptom. Additional features include lactic acidosis, muscle weakness and/or myoglobinuria. Defects in MTCYB are also found in cases of exercise intolerance accompanied by deafness, mental retardation, retinitis pigmentosa, cataract, growth retardation, epilepsy (multisystem disorder).
Cardiomyopathy, infantile histiocytoid (CMIH):
A heart disease characterized by the presence of pale granular foamy histiocyte-like cells within the myocardium. It usually affects children younger than 2 years of age, with a clear predominance of females over males. Infants present with dysrhythmia or cardiac arrest. The clinical course is usually fulminant, sometimes simulating sudden infant death syndrome.
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.

Heidari, M. M., Ebrahimi, F., Ardakani, Z. S., Mirzaei, M., Mirhosseini, S., & Khatami, M. (2023). Molecular Study of Nucleotide Changes of ATPase6 and MT-CYB Genes in the Mitochondrial Genome of Patients with Familial Adenomatous Polyposis (FAP). Journal of Shahid Sadoughi University of Medical Sciences.

Gromenko, Y. Y., Galimov, K. S., Gilyazova, I. R., Galimova, E. F., Bulygin, K. V., Ryagin, S. N., ... & Pavlov, V. N. (2023). Single nucleotide polymorphism rs527236194 of the cytochrome B gene (MT-CYB) is associated with alterations in sperm parameters. Molecular Biology Reports, 50(12), 10131-10136.

Finsterer, J., & Ghosh, R. (2023). Effective treatment of choreaballism due to an MT‐CYB variant with haloperidol, tetrabenazine, and antioxidants. Clinical Case Reports, 11(6), e7592.

Saleh Jaweesh, M., Hammadeh, M. E., Dahadhah, F. W., Al Zoubi, M. S., & Amor, H. (2022). Association between the single nucleotide variants of the mitochondrial cytochrome B gene (MT-CYB) and the male infertility. Molecular Biology Reports, 49(5), 3609-3616.

Lo Faro, V., Nolte, I. M., Ten Brink, J. B., Snieder, H., Jansonius, N. M., & Bergen, A. A. (2021). Mitochondrial genome study identifies association between primary open-angle glaucoma and variants in MT-CYB, MT-ND4 genes and haplogroups. Frontiers in Genetics, 12, 781189.

Zarrouk, S., Finsterer, J., Mehri, S., Ourda, F., Arab, S. B., & Boussada, R. (2021). Dilated Cardiomyopathy due to the Novel MT-CYB Missense Mutation m. 14757T> C. Journal of Medical Cases, 12(11), 455.

Alston, C. L., Blakely, E. L., McFarland, R., & Taylor, R. W. (2020). The m. 15043G> A MT-CYB variant is not a pathogenic mtDNA variant. Journal of the Neurological Sciences, 417.

Liu, J., Lu, Z., Yuan, C., Wang, F., & Yang, B. (2020). Phylogeography and phylogenetic evolution in Tibetan sheep based on MT-CYB sequences. Animals, 10(7), 1177.

Ghosh, R., Dubey, S., Chatterjee, S., Finsterer, J., Biswas, R., Lahiri, D., & Ray, B. K. (2020). Primary hypoparathyroidism and multiple neuraxial involvement in mitochondrial disorder due to the variant m. 15043G> A in MT-CYB. Journal of the Neurological Sciences, 414.

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

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