VPREB1

Description

The VPREB1 (V-set pre-B cell surrogate light chain 1) is a protein-coding gene located on chromosome 22.

VPREB1 encodes for the Immunoglobulin iota chain protein, also known as CD179A. This protein is found specifically in early B cell development, namely, in proB and early preB cells. VPREB1 is a 126 amino acid protein with a molecular weight of 16-18 kDa. It has a structure similar to an Ig V domain but lacks the final beta-strand (beta7). Instead, it has a carboxyl terminal end with no known homology to other proteins. VPREB1 associates with CD179b (lambda5 or lambda-like), forming a structure resembling an Ig light chain, known as the surrogate light chain or pseudo light chain. In this complex, the incomplete V domain of VPREB1 is complemented by the extra beta7 strand of CD179b. On the surface of early preB cells, the VPREB1/CD179b surrogate light chain forms a complex with the membrane-bound Ig mu heavy chain and the signal transducers CD79a/CD79b. This complex, known as the preB cell receptor (preBCR), is similar to a B cell receptor. While no human diseases or pathologies directly linked to VPREB1 have been identified, deficiencies in other components of the preB cell receptor, such as CD179b, Ig mu heavy chain, and CD79a, have been shown to significantly impair B cell development and lead to agammaglobulinemia in humans.

VPREB1 associates with the Ig-mu chain to form a molecular complex found on the surface of pre-B-cells. This complex likely plays a role in regulating Ig gene rearrangements during the initial stages of B-cell development.

VPREB1 is also known as CD179a, IGI, IGVPB, VPREB.

Associated Diseases

• Okt4 epitope deficiency
• neutropenia, severe congenital, 2, autosomal dominant
• combined immunodeficiency with skin granulomas
• common variable immunodeficiency
• immunodeficiency 19
• severe combined immunodeficiency due to IKK2 deficiency
• combined immunodeficiency due to partial RAG1 deficiency
• periodontal disorder