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CCL4

C-C motif chemokine ligand 4 (CCL4), also known as macrophage inflammatory protein 1-beta (MIP-1β), is a small belonging to the CC chemokine subfamily. It is a 92-amino-acid protein encoded by the CCL4 gene on chromosome 17 in humans, featuring a characteristic and capable of forming homodimers. Secreted primarily by immune cells such as , T cells, and dendritic cells in response to mitogenic or antigenic stimuli, CCL4 acts as a chemoattractant for monocytes, T lymphocytes, and natural killer cells, binding primarily to the receptors and CCR1. CCL4 plays a key role in inflammatory and immune responses, orchestrating leukocyte recruitment to sites of or injury. It is a potent suppressor of HIV-1 by blocking the receptor, which the virus uses for cellular entry. Dysregulated expression of CCL4 is implicated in various pathologies, including , , cancer progression, and autoimmune diseases, where it contributes to chronic inflammation and tissue remodeling.

Discovery and nomenclature

Historical discovery

CCL4, known initially as macrophage inflammatory protein-1β (MIP-1β), was discovered in 1988 through biochemical purification from supernatants of lipopolysaccharide (LPS)-activated murine macrophages. Researchers resolved the previously identified MIP-1 doublet into two distinct components, MIP-1α and MIP-1β, with the latter cloned from a cDNA library derived from the RAW 264.7 murine macrophage cell line. The cloning utilized synthetic oligonucleotide probes designed from partial N-terminal amino acid sequences of the purified 8-kDa protein, revealing a 92-amino-acid precursor with a 23-residue signal peptide. This work established MIP-1β as a novel proinflammatory cytokine secreted by activated macrophages. The homolog of MIP-1β was first cloned in 1988 as the ACT-2 gene product from a of phytohemagglutinin-activated human T cells, using differential hybridization to identify activation-inducible transcripts. This identified a 92-amino-acid encoding a secreted protein, confirmed by expression in baculovirus-infected cells. In 1990, further characterization linked ACT-2 to MIP-1β, demonstrating approximately 70% and sequence identity to the murine form, with the gene mapped to chromosome 17q11-q12 via . These studies, building on amplification from activated human T-cell RNA, solidified CCL4's identity as the human MIP-1β. Early functional assays in the revealed MIP-1β's potent chemotactic activity toward multiple immune cell types, underscoring its role in . In vitro microchemotaxis experiments demonstrated attraction of monocytes and CD4+ T lymphocytes, particularly naive CD45RA+ subsets, while showing differential effects compared to MIP-1α on CD8+ T cells. Additional studies confirmed its chemoattractant properties for natural killer () cells, promoting their migration in Boyden chamber assays and contributing to antiviral and antitumor responses. These findings highlighted MIP-1β's selectivity in recruiting effector cells during immune activation.

Nomenclature and synonyms

The official for CCL4, as designated by the International Union of Pharmacology (IUPHAR), is C-C motif ligand 4, with the approved gene symbol CCL4. This gene symbol was previously known as SCYA4, reflecting earlier conventions for small subfamily A genes. Common synonyms for the protein include inflammatory protein-1β (MIP-1β), activation-2 (ACT-2), activation gene-1 (LAG-1), and hematopoietic cell 21 (HC21). CCL4 belongs to the CC chemokine subfamily, also known as the β subfamily, characterized by the presence of two adjacent cysteine residues in the conserved N-terminal that forms bonds critical to its structure. This classification distinguishes it from other chemokine subfamilies, such as CXC (α), where a single separates the first two cysteines. The systematic naming of CCL4 as C-C chemokine ligand 4 originated from a 2000 proposal by the Chemokine Nomenclature Subcommittee of the International Union of Immunological Societies, which standardized chemokine ligands to replace historical names like MIP-1β and promote consistency across the field.80165-X) This renaming effort assigned sequential numbers to members of the CC subfamily (CCL1 through CCL28), with MIP-1β designated as CCL4 based on its sequence and functional similarities to other inflammatory .80165-X)

Genetics and expression

Genomic organization

The CCL4 gene is located on the long arm of human chromosome 17 at cytogenetic band 17q12, with precise genomic coordinates spanning 36,103,827 to 36,105,614 on the GRCh38 assembly. It resides within a on chromosomal region 17q11.2-q21, which encompasses related genes such as CCL3 and CCL3L1. The gene structure is compact, covering approximately 1.8 kb and comprising three exons separated by two introns, with the coding sequence distributed across all exons. The promoter region immediately upstream of exon 1 features binding sites for the transcription factors and AP-1, enabling rapid and inducible transcriptional activation in response to proinflammatory stimuli. CCL4 has two functional paralogs, CCL4L1 and CCL4L2, arising from events and located in close proximity within the same chromosomal cluster; these paralogs exhibit high sequence similarity to CCL4 and display copy number variations (typically 1-6 copies per diploid ) that modulate aggregate production levels across individuals. Such copy number variations are notably higher in non-human primates than in humans, highlighting lineage-specific evolutionary dynamics in this . Evolutionarily, the CCL4 gene is highly conserved among mammals, with orthologs in species including (Ccl4), reflecting ancient duplication and selection pressures in the chemokine superfamily; the human and mouse protein sequences share substantial identity, underscoring functional preservation across vertebrates.

Gene expression patterns

The CCL4 gene is primarily transcribed in activated immune cells, with prominent expression in T lymphocytes, particularly CD8+ subsets compared to CD4+ cells, as well as monocytes, macrophages, cells, and dendritic cells. In resting states, these cells exhibit minimal CCL4 production, but activation triggers robust upregulation, reflecting its role as an inducible . Expression of CCL4 is strongly induced by mitogenic stimuli such as phytohemagglutinin (PHA) and phorbol myristate acetate (PMA), which activate T cells and monocytes, leading to increased mRNA and protein levels within hours. Cytokines including interferon-gamma (IFN-γ) and tumor necrosis factor-alpha (TNF-α) further enhance transcription in macrophages and other producers through pathways involving NF-κB and MAPK signaling. Antigen-specific immune responses, such as those during viral infections, also drive CCL4 production in CD4+ and CD8+ T cells, contributing to coordinated leukocyte recruitment. In terms of tissue distribution, CCL4 shows elevated expression in lymphoid organs like the and lymph nodes, as well as the and sites of , where immune activation is prominent. Constitutive levels remain low in the and liver under homeostatic conditions, though inducible expression can occur in response to local stressors. Blood and bone marrow also display moderate baseline expression, primarily from resident immune populations. Post-transcriptional regulation of CCL4 involves microRNAs, notably miR-125b, which binds the 3' untranslated region of CCL4 mRNA to suppress its expression in monocytes and T cells, with reduced miR-125b levels linked to age-related increases in CCL4.

Protein structure and biochemistry

Primary and tertiary structure

The CCL4 protein is initially synthesized as a 92-amino-acid precursor that includes a 23-amino-acid N-terminal signal peptide, which is cleaved during post-translational processing to produce the mature polypeptide consisting of 69 amino acids and having a molecular weight of approximately 7.6 kDa. The primary sequence of mature CCL4 exhibits the conserved CC motif, characterized by adjacent residues at positions 11 and 12 that form intramolecular bonds with cysteines at positions 35 and 51 (Cys¹¹–Cys³⁵ and Cys¹²–Cys⁵¹), essential for maintaining integrity. The tertiary structure comprises a disordered N-terminal region, a short 3₁₀ , an antiparallel three-stranded β-sheet forming the core, and a C-terminal α- that packs against the β-sheet, with the bonds linking the N-loop to the β3 strand and C-terminal helix. X-ray crystallography has revealed that CCL4 adopts a dimeric arrangement in its (PDB: 2X6L), where the dimer interface is mediated by hydrogen bonding between the N-terminal loops of two , forming a six-stranded antiparallel β-sheet; however, in , CCL4 predominates as a at low concentrations but assembles into dimers and extends into reversible, rod-shaped higher-order polymers at elevated concentrations. CCL4 possesses biophysical characteristics including an acidic (pI) of approximately 4.5, thermal and pH stability within the range of 6–8 that supports its physiological roles, and surface-exposed clusters of basic residues (such as Arg¹⁸, Lys⁴⁵, and Arg⁴⁶) that facilitate electrostatic interactions with glycosaminoglycans.

Post-translational modifications

CCL4 undergoes post-translational proteolytic processing primarily at the , which modulates its . The full-length mature form of CCL4, consisting of 69 starting with Ala-Pro, is secreted by activated immune cells such as monocytes and T cells. CD26/dipeptidyl-peptidase IV (DPP-IV) cleaves the N-terminal Ala-Pro after secretion, generating the truncated CCL4(3-69) form. This processing occurs in cytokine-stimulated peripheral blood lymphocytes and reduces interactions with glycosaminoglycans (GAGs), thereby shortening the of the . The truncated CCL4(3-69) retains binding and signaling capacity through but exhibits diminished chemotactic potency compared to the full-length form, which is more effective at eliciting CCR5-mediated responses in T cells and macrophages. However, the truncation expands receptor specificity, enabling agonistic activity at CCR1 and CCR2b, which promotes of monocytes, myeloid-derived suppressor cells, immature dendritic cells, and lymphocytes. This shift preserves anti-HIV-1 suppressive effects while altering hematopoietic interactions, as the full-length form enhances colony formation more potently. Inhibition of CD26/DPP-IV prevents this truncation, maintaining the full-length CCL4's profile. Additional proteolytic cleavage by insulin-degrading enzyme (IDE) targets the of CCL4, leading to its degradation and reduced under physiological conditions. This process diminishes chemotactic activity and facilitates clearance, contributing to regulation of CCL4 levels during . Polymerization into higher-order oligomers, while not a covalent modification, enhances CCL4 and for GAGs, indirectly influencing its localization and in tissues.

Biological functions

Chemotactic activity

CCL4, also known as macrophage inflammatory protein-1β (MIP-1β), serves as a potent chemoattractant for various immune cells, directing their migration to sites of inflammation or infection. It primarily targets CD4+ and CD8+ T cells, monocytes, eosinophils, basophils, and immature dendritic cells, eliciting responses at nanomolar concentrations (typically 1–10 nM). This selective attraction is mediated through binding to the receptor CCR5, which is expressed on these cell types, thereby facilitating immune surveillance and response coordination. The chemotactic mechanism of CCL4 relies on the establishment of spatial gradients that guide cell movement. Upon secretion, CCL4 binds to glycosaminoglycans (GAGs) on the endothelial surface, immobilizing it and forming stable haptotactic cues that promote leukocyte adhesion and transmigration across the vascular wall. This GAG-dependent immobilization enhances the local presentation of CCL4, converting soluble signals into substrate-bound gradients that support directed, integrin-mediated migration on the extracellular matrix. Studies using GAG-binding mutants have demonstrated that disruption of this interaction abolishes in vivo recruitment while preserving in vitro chemotaxis, underscoring its role in physiological contexts. In vitro assessments of CCL4's chemotactic potency commonly employ Boyden chamber assays, where peak of responsive cells, such as monocytes and T cells, occurs at concentrations of 10–100 ng/mL (approximately 1.25–12.5 nM). These assays reveal dose-dependent responses, with optimal activity reflecting physiological relevance.

Inflammatory roles

CCL4 exerts pro-inflammatory effects by facilitating the recruitment of immune cells, including Th1 cells and macrophages, to sites of and tissue damage. As a ligand for , CCL4 promotes the of Th1-polarized + T cells across endothelial barriers during inflammatory responses, enhancing adaptive immunity against . Similarly, CCL4 drives the influx of inflammatory monocytes and macrophages to infected tissues, as demonstrated in models of viral infections where type I signaling upregulates CCL4 expression to constrain spread. This recruitment amplifies local inflammation; for instance, CCL4, along with related , contributes to the in severe infections by inducing the release of pro-inflammatory mediators such as TNF-α and IL-1β from macrophages. Beyond acute inflammation, CCL4 participates in homeostatic processes, including the maintenance of T cell homeostasis and viral clearance. In lymphoid tissues, CCL4 secreted by regulatory T cells modulates T cell proliferation and suppresses excessive activation, thereby preserving immune balance. Notably, CCL4 aids in HIV suppression by competing with the virus for binding to the CCR5 co-receptor on CD4+ T cells, reducing viral entry and promoting clearance in infected individuals. This natural antagonistic role highlights CCL4's dual function in limiting certain viral pathologies while supporting overall T cell homeostasis. CCL4 exhibits functional and with CCL3 (MIP-1α), particularly through the formation of CCL3•CCL4 heterodimers that display enhanced chemotactic potency compared to individual homodimers, as observed in activated human T s and s. In models, elevated levels of both CCL3 and CCL4 in correlate with disease severity, where their cooperative action amplifies and T infiltration, exacerbating . However, negative regulation occurs at high concentrations; prolonged exposure to CCL4 induces homologous desensitization of receptors on target s, thereby attenuating further signaling and limiting excessive inflammatory responses to prevent tissue damage. This self-limiting mechanism underscores CCL4's role in balancing pro- and dynamics.

Molecular interactions

Receptor binding

CCL4, also known as macrophage inflammatory protein-1β (MIP-1β), primarily binds to the G-protein-coupled receptor with high affinity, characterized by a dissociation constant (Kd) of approximately 4 . This interaction involves the N-terminal domain of CCL4 engaging the extracellular loops (ECLs), particularly ECL2 and ECL3, of , facilitating receptor activation through insertion of the chemokine's globular core into the transmembrane helical bundle. In addition to CCR5, CCL4 exhibits weak binding to CCR1, with affinity reduced by approximately 100-fold compared to CCR5, and minimal interaction with CCR3, limiting its functional activity on these receptors. CCL4 shows no binding or activity on , a CXC that recognizes distinct ligands such as CXCL12. Binding kinetics of CCL4 to CCR5 are enhanced in its dimeric form, which displays higher due to cooperative interactions that stabilize receptor engagement, particularly in the context of glycosaminoglycan ()-bound oligomers on cell surfaces. , such as , play a crucial role by facilitating the immobilization and haptotactic presentation of CCL4 on endothelial surfaces, promoting leukocyte trans-endothelial migration through sustained receptor signaling. Upon binding to CCR5, CCL4 induces Gαi-mediated signaling, leading to pertussis toxin-sensitive activation of downstream pathways including intracellular calcium flux and phosphorylation of extracellular signal-regulated kinase (ERK). These events contribute to rapid cellular responses such as , though detailed pathway propagation is addressed in functional contexts.

Protein-protein interactions

CCL4 engages in several key protein-protein interactions that modulate its localization, stability, and activity, independent of receptor engagement. One prominent interaction is its tendency to form both homodimers and heterodimers with the closely related CCL3 (MIP-1α). These dimers are stabilized by salt bridges, notably between aspartate 27/glutamate 67 and arginine 46/arginine 48, as well as hydrogen bonds such as that between aspartate 6 and serine 33. This dimerization interface is conserved between CCL3 and CCL4 due to their high sequence similarity, and the resulting complexes enhance the stability in physiological conditions, preventing rapid degradation and facilitating sustained presentation at inflammatory sites. Heterodimers of CCL3 and CCL4 are naturally secreted by activated monocytes and lymphocytes, representing one of the earliest identified examples of functional chemokine heteromers. In addition to self-association, CCL4 interacts with glycosaminoglycans (GAGs), particularly and , which anchor the to extracellular matrices and cell surfaces. These interactions are mediated primarily by clusters of residues in the 20s loop and 40s loop of CCL4, including arginine 18 and 46, which form electrostatic bonds with the negatively charged sulfate groups on the chains. studies have confirmed the involvement of nearby residues such as 45 and serine 47 in this binding, underscoring the role of the C-terminal region's patch. By immobilizing CCL4 on proteoglycan-bearing surfaces, these interactions promote haptotactic gradients that guide leukocyte migration without relying on soluble . Furthermore, CCL4 contributes to antiviral defense by inhibiting HIV-1 entry through steric hindrance of the glycoprotein gp120's interaction with on target cells, a mechanism shared among .

Role in physiology and disease

Normal physiological roles

CCL4 contributes to immune through its constitutive low-level expression in various tissues, including mucosal surfaces, where it facilitates baseline trafficking of T cells and other leukocytes. As a chemoattractant for T lymphocytes, monocytes, and , CCL4 supports routine immune surveillance by promoting the migration of these cells to mucosal sites without inducing overt . This function helps maintain steady-state immune patrolling, ensuring rapid responsiveness to potential threats in barrier tissues. In processes, CCL4 plays a key role in recruiting macrophages to injury sites, aiding in debris clearance and tissue repair. By interacting with receptors such as , CCL4 draws monocytes and macrophages to the wound bed, where they orchestrate the transition from inflammation to proliferation phases. CCL4 supports aspects of development, particularly in the maturation and migration of natural killer (NK) cells within the microenvironment. It induces calcium mobilization in NK cells, contributing to their functional priming during development. Furthermore, CCL4 regulates thymic emigration by attracting maturing T cells, ensuring proper population of peripheral lymphoid organs for immune . In viral defense, CCL4 acts as a natural antagonist to CCR5-tropic HIV-1 entry in non-infected cells by competitively binding to the coreceptor, thereby blocking viral attachment and fusion. This protective mechanism underscores CCL4's role in innate antiviral immunity, preventing pathogen spread in healthy tissues.

Involvement in pathologies

CCL4, also known as macrophage inflammatory protein-1β (MIP-1β), plays a significant role in . In chronic -1 infection, CCL4 levels are elevated in lymph nodes and plasma, contributing to sustained immune activation and altered environments that facilitate viral persistence. Additionally, polymorphisms in the CCL4L1 gene, which encodes a variant of CCL4, influence susceptibility; lower copy numbers of CCL4L1 (less than 2 copies) are associated with a 75% increased of HIV acquisition, faster progression to AIDS (approximately twofold acceleration), and heightened mother-to-child (10- to 13-fold in low-copy pairs). In autoimmune diseases, CCL4 overexpression drives inflammation in (RA) and (MS). In RA, elevated CCL4 concentrations in , produced by T cells and monocytes, correlate with disease activity and promote joint inflammation by recruiting immune cells and inducing production from synovial tissues. In MS, CCL4 contributes to blood-brain barrier (BBB) disruption by inducing inflammatory signaling in neurovascular endothelium via , increasing permeability and facilitating immune cell infiltration into the . CCL4 exhibits dual roles in cancer, promoting metastasis in some contexts while exerting antitumor effects in others. In breast cancer, CCL4 facilitates bone metastasis by mediating interactions between tumor cells and CCR5-expressing fibroblasts in the bone microenvironment, enhancing tumor cell adhesion and survival. In oral squamous cell carcinoma, CCL4 induces vascular endothelial growth factor C (VEGF-C) expression through the JAK2/STAT3 pathway and miR-195-3p regulation, promoting lymphangiogenesis and metastatic spread to lymph nodes. Conversely, in certain tumors, CCL4 recruits natural killer (NK) cells via CCR5, enhancing antitumor immunity; for instance, CCL4 released by M1-polarized tumor-associated macrophages supports NK cell infiltration and cytotoxicity. Beyond these, recent research highlights CCL4's involvement in osteoarthritis (OA), coronary artery disease (CAD), and aging-related vascular defects. In OA, CCL4/CCR5 signaling regulates apoptosis and production, accelerating degradation and disease progression. In CAD, higher circulating CCL4 levels, as evidenced by , increase risk by promoting infiltration and atherosclerotic plaque formation. During aging, elevated CCL4 activates and endothelial , leading to angiogenic insufficiency and impaired vascular repair.

Therapeutic and research applications

Potential as biomarker

CCL4, also known as macrophage inflammatory protein-1β (MIP-1β), has emerged as a potential biomarker for diagnosing and monitoring immune-mediated diseases due to its role in recruiting immune cells to sites of inflammation. Serum levels of CCL4 are significantly elevated in patients with active rheumatoid arthritis (RA) compared to healthy controls, with studies reporting higher circulating concentrations in untreated early RA cohorts using bead-based immunoassays. Similarly, in HIV infection, plasma CCL4 levels are increased in affected individuals relative to uninfected controls, correlating with immune activation and disease presence, as measured by multiplex biomarker panels. Standardized enzyme-linked immunosorbent assay (ELISA) kits, such as those from R&D Systems, enable reliable quantification of CCL4 in serum and plasma with a typical assay range of 31.2–2,000 pg/mL and sensitivity of 11 pg/mL for serum and plasma samples, facilitating clinical assessment. In prognostic applications, elevated CCL4 expression in the has been associated with adverse outcomes in , particularly increased risk of . Genetic variations in the , including polymorphisms, contribute to progression and metastatic potential by enhancing signaling that promotes tumor cell and fibroblast activation. Although post-2023 meta-analyses specifically on CCL4 in are limited, earlier comprehensive analyses and functional studies underscore its role in predicting poor survival and through interactions with CCR5-expressing stromal cells. CCL4 also shows promise for monitoring therapeutic responses, particularly in inflammatory conditions like (IBD), where its levels may track inflammation dynamics during ; for example, serum macrophage inflammatory protein-1β (CCL4) levels have been identified as sensitive markers for response in patients treated with . Genetic variants, such as copy number variations (CNV) in the related CCL4L1 gene, serve as predisposing markers for disease susceptibility, notably in where low CCL4L1 copy numbers are linked to accelerated progression and higher risk. In IBD, CCL4 expression correlates with immune cell infiltration. Despite these utilities, challenges in using CCL4 as a include inter-individual variability arising from CNV in CCL4L1 and related genes like CCL3L1, which can alter baseline expression and complicate threshold-based diagnostics. Accurate assessment often requires with CCL3 (MIP-1α) to account for functional and shared genetic , as their combined copy number profiles better predict associations than CCL4 alone. These factors highlight the need for integrated genetic and proteomic approaches to enhance biomarker reliability.

Drug targeting and inhibitors

Pharmacological strategies to target CCL4 primarily focus on its receptor , using s that prevent CCL4-induced signaling. Maraviroc, the first CCR5 approved by the FDA in 2007, blocks the binding of CCL4 (along with CCL3 and ) to CCR5, thereby inhibiting downstream signaling pathways essential for immune cell recruitment and HIV-1 entry. This indirect modulation of CCL4 activity has proven effective in treating CCR5-tropic HIV-1 infections when combined with other antiretrovirals. In addition to its antiviral role, Maraviroc has been repurposed for , where it disrupts CCL4-mediated inflammation and tumor progression; preclinical studies show it inhibits and recruitment in models. Reviews as of 2024 have explored its potential in various cancers, including combinations with chemotherapeutics. Direct inhibition of CCL4 through neutralizing antibodies represents another key approach, particularly for autoimmune and inflammatory diseases. Monoclonal antibodies targeting CCL4, often as part of broader CC chemokine neutralization, have demonstrated preclinical efficacy in reducing . For instance, a humanized monoclonal antibody that binds and neutralizes CCL4 (along with CCL3 and ) restricts splenocyte egress from lymphoid tissues and limits leukocyte infiltration, significantly ameliorating symptoms in models of by decreasing and tissue damage. These antibodies, such as those in early development for conditions like , operate by preventing CCL4 from engaging receptors or forming bioactive complexes, offering a targeted means to dampen excessive chemokine-driven responses without broad . Emerging small-molecule inhibitors aim to disrupt CCL4's structural and interactive properties, including dimerization and (GAG) binding. Peptide mimetics designed to mimic CCL4's interface have been investigated to interfere with its dimer formation, which is critical for stabilizing higher-order oligomers that enhance its pro-inflammatory activity; analogous approaches for related CC chemokines like have shown promise in blocking oligomerization and reducing chemotactic potency. Additionally, GAG competitors, such as sulfated or synthetic analogs, prevent CCL4 immobilization on endothelial surfaces, thereby disrupting haptotactic gradients that guide ; research highlights this strategy's potential in inhibiting across chemokine families, including CCL4, with ongoing efforts to develop orally bioavailable compounds. Gene therapy targeting CCL4L1, a paralog of CCL4 with variable copy numbers, holds potential for enhancing resistance by increasing endogenous CCL4 production to downregulate expression. Higher CCL4L1 copy numbers are associated with slower progression due to elevated levels that compete for binding, reducing viral entry. While CRISPR-based editing has advanced in gene therapy, such as targeting or viral genomes, specific applications to amplify CCL4L1 copies face challenges including off-target edits and ensuring physiological regulation to avoid excessive inflammation; preclinical models underscore the need for precise control to balance protection with immune .