Abcam, ab220914, Anti-COQ2 antibody

Size: 100µL
Rabbit Polyclonal COQ2 antibody. Suitable for WB, ICC/IF and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human COQ2 aa 250-350.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:WB, ICC/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human COQ2 aa 250-350. The exact immunogen used to generate this antibody is proprietary information.Q96H96

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7.2Preservative: 0.02% Sodium azideConstituents: PBS, 40% Glycerol (glycerin, glycerine), Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
COQ2 also known as PDSS1 is a protein involved in the biosynthesis of coenzyme Q10 (CoQ10) an important molecule in cellular energy production. COQ2 has a molecular weight of approximately 39 kDa and is expressed mainly in the mitochondria of most tissues with high levels in energy-demanding tissues like the heart kidneys and liver. COQ2 serves as a polyprenyl transferase enzyme facilitating the transfer of a polyprenyl group to para-hydroxybenzoate a critical step in the production of CoQ10.
Biological function summary
COQ2 plays a central role in producing CoQ10 a fundamental component of the mitochondrial electron transport chain. COQ2 is part of the larger CoQ biosynthetic complex which includes several other COQ proteins. These proteins work together to ensure the correct synthesis and regulation of CoQ10. This collective action helps maintain cellular energy homeostasis and protects against oxidative stress by scavenging free radicals.
Pathways
COQ2 is an essential player in the coenzyme Q biosynthesis pathway and more generally in the oxidative phosphorylation pathway. Interaction with other proteins such as COQ3 and COQ9 further enhances its function in these pathways. COQ2's contribution to the electron transport chain allows efficient ATP production essential for overall cellular metabolism.
Mutations in the COQ2 gene have been linked to CoQ10 deficiency manifesting as various clinical conditions like nephrotic syndrome and cerebellar ataxia. Altered COQ2 function may impact COQ8 another affinity protein within the coenzyme Q biosynthesis pathway aggravating these conditions due to compromised energy production in affected tissues. Understanding these associations aids in deciphering how COQ2 mutations contribute to mitochondrial-related diseases.