Abcam, ab235084, Anti-PEX26 antibody

Size: 100µL
Rabbit Polyclonal PEX26 antibody. Suitable for WB, IHC-P and reacts with Human samples. Cited in 2 publications. Immunogen corresponding to Recombinant Fragment Protein within Human PEX26 aa 1-200.
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 PEX26 aa 1-200. The exact immunogen used to generate this antibody is proprietary information.Q7Z412

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Protein G, Purification notes-Purity >95%, Storage buffer-pH: 7.4Preservative: 0.03% Proclin 300Constituents: PBS, 50% 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.
PEX26 also known as Peroxin 26 is a protein critical for peroxisomal function. It has a molecular mass of approximately 33 kDa. PEX26 is localized in the peroxisomal membrane and is mainly expressed in various tissues including liver kidney and brain. Its role is important in the peroxisome import machinery where it anchors the PEX1-PEX6 complex to the peroxisome membrane enabling the transport and maintenance of matrix proteins necessary for peroxisome function.
Biological function summary
The importance of PEX26 lies in its role as part of the peroxisome biogenesis complex. It anchors PEX1 and PEX6 which are essential components for ATPase activity important for peroxisome assembly and function. This tightly regulated process ensures that peroxisomes function correctly in metabolic pathways such as fatty acid β-oxidation and reactive oxygen species detoxification.
Pathways
PEX26 is instrumental in the peroxisomal matrix protein import pathway. This pathway includes the interaction between PEX1 and PEX6 where PEX26 is an anchor within the membrane structure. The pathway's proper function is essential to cellular lipid metabolism and detoxification processes linking it to proteins such as PEX5 that acts as a receptor for matrix proteins targeting peroxisomes.
Defects in PEX26 often associate with peroxisome biogenesis disorders such as Zellweger Spectrum Disorders (ZSD). These disorders result from improper peroxisome function due to mutations in the PEX26 gene leading to various metabolic dysfunctions. PEX26 mutations are especially significant in Zellweger syndrome a severe form of ZSD. The inability to correctly bind PEX1 and PEX6 leads to defective peroxisomal assembly critically impacting cellular health and leading to clinical manifestations associated with these disorders.