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AEBP1
AEBP1 Full Name
AE binding protein 1
AEBP1 Introduction
AE binding protein 1 (AEBP1), which encodes the secreted aortic carboxypeptidase-like protein (ACLP), is a critical extracellular matrix (ECM)-associated protein. Structurally equipped with a carboxypeptidase domain and a discoidin-like domain, AEBP1 functions primarily as a fundamental regulator of tissue remodeling and ECM architecture. Once secreted into the extracellular space by fibroblasts and osteoprogenitors, it physically binds to fibrillar collagens and actively assists in collagen polymerization and crosslinking. By directing the proper assembly of the collagen network, AEBP1 ensures the mechanical strength, elasticity, and structural integrity of connective tissues throughout the body, particularly in the skin, vasculature, and skeletal system. Furthermore, it acts as a vital signaling modulator, enhancing Wnt/β-catenin and transforming growth factor-beta (TGF-β) pathways to regulate fibroblast activation, adipogenesis, and normal osteoblast differentiation.
Clinically, the profound importance of AEBP1 is best illustrated by the severe consequences of its genetic disruption. Biallelic loss-of-function mutations in the AEBP1 gene are the direct cause of classic-like Ehlers-Danlos syndrome type 2 (clEDS2). In the absence of functional AEBP1, collagen fibers fail to assemble correctly, leading to a severely fragmented and weakened ECM. Consequently, patients present with hallmark connective tissue defects, including severe skin hyperextensibility, joint hypermobility, abnormal wound healing, and early-onset osteoporosis due to severely impaired osteoblast signaling.
Conversely, the pathological overexpression of AEBP1 is a major driver of fibrosis and cancer progression. In the dense tumor microenvironment of various highly aggressive solid tumors (such as oral squamous cell carcinoma and colorectal cancer), AEBP1 is heavily upregulated by cancer-associated fibroblasts (CAFs). This hyperactive stromal expression forces excessive collagen deposition and dramatically increases ECM mechanical stiffness. This dense, fibrotic physical barrier not only promotes tumor cell invasion but actively excludes CD8+ cytotoxic T-cells from penetrating the tumor core, driving profound immune evasion. Consequently, AEBP1 represents a highly compelling therapeutic target: restoring its function is crucial for treating EDS, while targeting its stromal expression holds immense promise for overcoming physical barriers in cancer immunotherapy.
Figure 1. Schematic illustration of the mechanism by which CAF-derived AEBP1 induces T cell dysfunction in tumors. (Source: Wang X, et al. 2025)
Alternate Names for AEBP1
AEBP1; AE binding protein 1; ACLP; adipocyte enhancer-binding protein 1; aortic carboxypeptidase-like protein;
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