Mouse anti-Human sapiens (Human) PSMB8 Monoclonal Antibody

Function

The proteasome is a multicatalytic proteinase complex which is characterized by its ability to cleave peptides with Arg, Phe, Tyr, Leu, and Glu adjacent to the leaving group at neutral or slightly basic pH. The proteasome has an ATP-dependent proteolytic activity. This subunit is involved in antigen processing to generate class I binding peptides. Replacement of PSMB5 by PSMB8 increases the capacity of the immunoproteasome to cleave model peptides after hydrophobic and basic residues. Involved in the generation of spliced peptides resulting from the ligation of two separate proteasomal cleavage products that are not contiguous in the parental protein (PubMed:27049119). Acts as a major component of interferon gamma-induced sensitivity. Plays a key role in apoptosis via the degradation of the apoptotic inhibitor MCL1. May be involved in the inflammatory response pathway. In cancer cells, substitution of isoform 1 (E2) by isoform 2 (E1) results in immunoproteasome deficiency. Required for the differentiation of preadipocytes into adipocytes

Subcellular Location

Cytoplasm; Nucleus

Disease Association

Proteasome-associated autoinflammatory syndrome 1 (PRAAS1) : An autosomal recessive autoinflammatory disorder characterized by early childhood onset of recurrent fever, joint stiffness and severe contractures of the hands and feet, and erythematous skin lesions with subsequent development of lipodystrophy and laboratory evidence of immune dysregulation. Accompanying features may include muscle weakness and atrophy, hepatosplenomegaly, and microcytic anemia. [The disease is caused by variants affecting the gene represented in this entry]

Subunit

(Microbial infection) Interacts with HIV-1 TAT protein

• Glynne R. et al. The major histocompatibility complex-encoded proteasome component LMP7: alternative first exons and post-translational processing. — Eur. J. Immunol. (1993)
• Beck S. et al. DNA sequence analysis of 66 kb of the human MHC class II region encoding a cluster of genes for antigen processing. — J. Mol. Biol. (1992)
• Glynne R. et al. A proteasome-related gene between the two ABC transporter loci in the class II region of the human MHC. — Nature (1991)
• Fruh K. et al. Alternative exon usage and processing of the major histocompatibility complex-encoded proteasome subunits. — J. Biol. Chem. (1992)
• Meinhardt T. et al. Different genomic structure of mouse and human Lmp7 genes: characterization of MHC-encoded proteasome genes. — Immunogenetics (1993)
• Beck S. et al. Evolutionary dynamics of non-coding sequences within the class II region of the human MHC. — J. Mol. Biol. (1996)
• Maksymowych W.P. et al. Sequence analysis of the HLA-linked LMP7 gene. (NOV-)
• Mungall A.J. et al. The DNA sequence and analysis of human chromosome 6. — Nature (2003)