Abcam, ab101990, Anti-Gigaxonin antibody

Size: 100µg
Rabbit Polyclonal Gigaxonin antibody. Suitable for IP and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Synthetic Peptide within Human GAN aa 50-100.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:IPSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Synthetic Peptide within Human GAN aa 50-100. The exact immunogen used to generate this antibody is proprietary information.Q9H2C0

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7 - 8 Preservative: 0.09% Sodium azide Constituents: Tris citrate/phosphate, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions-+4°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.
Gigaxonin also known as GAN or KLHL16 is a cytoskeletal protein with a mass of approximately 69 kDa. It functions as an E3 ubiquitin ligase adapter and is part of the BTB-Kelch protein family. By regulating protein degradation gigaxonin maintains cellular homeostasis. This protein is especially expressed in neural tissues including the brain and spinal cord. Through its role in controlling the turnover of intermediate filament proteins like vimentin gigaxonin plays an essential part in maintaining the structural integrity of cells.
Biological function summary
Gigaxonin plays an important role in the nervous system and supports healthy neuronal function. It functions in conjunction with other components as part of the ubiquitin-proteasome system complex ensuring the timely degradation of specific proteins. This collaboration ensures the removal of misfolded or damaged proteins preventing neuronal dysfunction. The regulation of intermediate filaments and microtubules by gigaxonin is also important for cellular architecture and intracellular transport which are key for proper neuronal activity.
Pathways
The protein is integral to both the cytoskeleton organization and protein degradation pathways. In these pathways gigaxonin interacts with proteins like tubulin and peripherin influencing microtubule dynamics and neuronal differentiation. By overseeing the balance between synthesis and degradation of cytoskeletal elements gigaxonin ensures neuron integrity and function. These pathways involving gigaxonin are needed for protein quality control and overall cellular health.
Gigaxonin mutations are linked to giant axonal neuropathy (GAN) a rare inherited neurodegenerative condition characterized by abnormally large axons and motor and sensory deficits. Aberrations in gigaxonin impair its ability to mediate the degradation of proteins such as neurofilaments leading to their accumulation. The linkage to GAN emphasizes the protein's role in maintaining neural health. Additionally abnormalities in gigaxonin function have implications for neurodegenerative diseases where intermediate filament accumulation presents a common feature.