Abcam, ab272616, Anti-NSUN3 antibody

Size: 100µL
Rabbit Polyclonal NSUN3 antibody. Suitable for IHC-P, WB and reacts with Human samples. Cited in 5 publications. Immunogen corresponding to Recombinant Fragment Protein within Human NSUN3 aa 100-250.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:IHC-P, WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human NSUN3 aa 100-250. The exact immunogen used to generate this antibody is proprietary information.Q9H649

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.
The NSUN3 protein also known as NOP2/Sun domain family member 3 functions as an RNA methyltransferase. It specifically catalyzes the methylation of cytosine in mitochondrial tRNA molecules which is a critical step for proper mitochondrial protein translation. NSUN3 is approximately 60 kDa in mass. It expresses mostly in tissues with high energy demands such as heart and muscle tissues due to its role in mitochondrial function.
Biological function summary
The function of NSUN3 is tightly linked to mitochondrial ribosome structure and translation. By methylating tRNA at cytosine-34 NSUN3 participates in the formation of the anticodon wobble position which is vital for accurate and efficient mitochondrial protein synthesis. It does not act alone as it often functions as part of larger mitochondrial translation complexes contributing to proper mitochondrial ribosomal assembly and function.
Pathways
NSUN3 is an integral component of the mitochondrial gene expression and oxidative phosphorylation pathways. It plays a significant role in regulating mitochondrial translation by directly modifying tRNA and interacts with other proteins like TRMT10C that also participate in this modification process. These pathways are important for maintaining mitochondrial energy metabolism and production of ATP within cells.
NSUN3 deficiency associates with mitochondrial disorders that manifest as systemic energy production failures due to impaired mitochondrial translation. These deficiencies often overlap with conditions like mitochondrial myopathy or lactic acidosis. Additionally the malfunction or absence of NSUN3 can disrupt interactions with other mitochondrial proteins like MTO1 further exacerbating mitochondrial dysfunction and leading to disease progression.