Invitrogen™ UBB (mutant) Monoclonal Antibody (83456)

Description

Reconstitute at 0.5 mg/mL in sterile PBS.

UBB (ubiquitin B) is a gene that encodes ubiquitin, a small regulatory protein that is ubiquitously expressed in eukaryotic cells. Ubiquitin plays a critical role in various cellular processes, including protein degradation, DNA repair, cell cycle regulation, and response to oxidative stress, by tagging proteins for degradation via the ubiquitin-proteasome system. Mutations in the UBB gene can lead to the production of defective ubiquitin proteins that can disrupt these essential cellular processes. One notable mutant form, UBB+1, is produced through a molecular process called frameshift mutation, which can occur during transcription. This mutant ubiquitin acts as a proteasome inhibitor rather than a substrate, leading to the accumulation of misfolded or damaged proteins. Such accumulation is associated with neurodegenerative diseases, including Alzheimer's disease and other forms of dementia, where impaired protein degradation contributes to disease pathology. Understanding the impact of UBB mutations, particularly UBB+1, is crucial for developing therapeutic strategies aimed at enhancing proteasome function and mitigating the effects of protein aggregation in neurodegenerative disorders. Research into UBB mutants also provides broader insights into the mechanisms of protein homeostasis and the potential for targeting the ubiquitin-proteasome system in various diseases.