SQSTM1 Human

Sequestosome 1 is a ubiquitin-binding protein that acts as a scaffold/adaptor protein. It is involved in the selective degradation of ubiquitinated proteins through autophagy, a process known as aggrephagy . The protein contains multiple domains that facilitate its interaction with various proteins and organelles, enabling it to bridge polyubiquitinated proteins and autophagosomes .

1. Autophagy: Sequestosome 1 is essential for the recruitment of ubiquitinated cargo proteins to autophagosomes. It interacts with microtubule-associated protein 1 light chain 3 (MAP1LC3), which is crucial for the formation of autophagosomes .
2. Signal Transduction: SQSTM1 regulates the activation of the NF-kB signaling pathway by acting as a scaffold for the assembly of signaling complexes. It interacts with tumor necrosis factor receptor-associated factor 6 (TRAF6) to mediate NF-kB activation in response to upstream signals .
3. Oxidative Stress Response: The protein is involved in the cellular response to oxidative stress by regulating the degradation of damaged proteins and organelles .

Mutations in the SQSTM1 gene are associated with several diseases, including:

• Paget’s Disease of Bone: A disorder characterized by abnormal bone remodeling, leading to bone pain, deformities, and fractures .
• Frontotemporal Dementia and/or Amyotrophic Lateral Sclerosis (FTD/ALS): Neurodegenerative disorders that affect cognitive and motor functions .

Recombinant Sequestosome 1 (Human) is produced using recombinant DNA technology, which involves the insertion of the human SQSTM1 gene into an expression system, such as bacteria or yeast, to produce the protein in large quantities. This recombinant protein is used in various research applications to study its structure, function, and role in disease processes.