Anti-RAG-1 Antibody

Descrição detalhada : V(D)J recombination-activating protein 1 (RAG-1) is also called RING finger protein 74. RAG-1 is a component of the RAG complex, which mediates the DNA cleavage phase during V(D)J recombination. As part of the RAG complex, RAG1 mediates the DNA-binding and catalyzes the DNA cleavage activities by introducing a double-strand breaks between the recombination signal sequences (RSS) and the adjacent coding segment. RAG-1 also acts as a E3 ubiquitin-protein ligase that mediates monoubiquitination of histone H3 as well as polyubiquitination of KPNA1. RAG-1 is expressed in maturing lymphoid cells. RAG-1 defects can cause disease states such as: combined cellular and humoral immune defects with granulomas (CHIDG), severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-negative/NK-cell-positive (T-B-NK+ SCID), Omenn syndrome (OS), and alpha/beta T-cell lymphopenia with gamma/delta T-cell expansion severe cytomegalovirus infection and autoimmunity (T-CMVA).
Sinónimos : V(D)J recombination-activating protein 1, RAG-1, RING finger protein 74
Armazenamento : +2°C to +8°C
Embalagem : 1X1EA