Target intelligence / Profile preview

V-set pre-B cell surrogate light chain 3 (VPREB3)

Target
VPREB3
Molecular classification
Immunoglobulin superfamily protein, Pre-B cell surrogate light chain
01

Overview

V-set pre-B cell surrogate light chain 3 (VPREB3) is a small glycoprotein expressed predominantly in early B cell precursors and germinal center B cells. It is a member of the immunoglobulin superfamily, structurally related to other surrogate light chain proteins such as VPREB1 and IGLL1, and typically functions as a component of the pre-B cell receptor complex. VPREB3 associates with immunoglobulin mu heavy chains in the endoplasmic reticulum, modulating their maturation and possibly their transport. While it is not deemed essential for B-cell development, its expression is a reliable marker of certain B-cell malignancies, serving as a valuable immunophenotypic biomarker for the diagnosis and classification of Burkitt lymphoma and some DLBCL subtypes, particularly those harboring c-MYC translocations. Outside hematopoietic tissues, VPREB3 is also found in Purkinje cells of the cerebellum and adrenal zona glomerulosa, but its function in these tissues remains unclear.

Other names
Pre-B lymphocyte protein 3Pre-B lymphocyte 3Pre-B lymphocyte gene 3Protein VPreB3VPREB38HS20N27C7-2UNQ355/PRO619
02

Mechanism of action

Null (No drugs target VPREB3 directly, so no mechanism of action is established).

03

Biological functions

B-cell development: Involved in early B-cell maturation.Pre-B cell receptor assembly: Associates with Ig mu chains during biosynthesis.Chaperone activity in the endoplasmic reticulum: Modulates immunoglobulin component processing.Possible mu chain transport
04

Disease associations

Cancer: Overexpressed and serves as a diagnostic marker in Burkitt lymphoma and certain subtypes of diffuse large B-cell lymphoma (DLBCL), especially those with c-MYC translocations.Immune/hematologic disorders: Associations with plasma fibronectin deficiency, congenital enteropathy due to enteropeptidase deficiency, and others.Other: Expression changes observed in some immune-related and neurological tissues
05

Biomarkers

Diagnostic marker for Burkitt lymphoma: Immunohistochemistry or gene expression can aid diagnosis or subtyping, especially to identify c-MYC–translocated lymphomas

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