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Mesangial cell

Mesangial cells are specialized that reside within the mesangium of the renal , comprising approximately 30–40% of all glomerular cells and originating from the metanephric . These cells, along with their associated , form the structural core of the , embedding between capillary loops and connecting to the via . Characterized as myofibroblast-like cells with contractile and phagocytic properties, they exhibit features similar to cells, enabling them to influence glomerular architecture and function. In terms of function, mesangial cells provide essential structural support to the glomerular capillary network, regulating the surface area available for filtration by contracting in response to factors such as angiotensin II. They also play a critical role in maintaining the filtration barrier through phagocytic clearance of macromolecules and debris, ensuring the permselectivity that allows passage of water and small solutes while restricting larger proteins. Additionally, these cells produce and regulate the mesangial matrix, which includes components like collagen IV, laminin, fibronectin, and proteoglycans, modulated by cytokines such as transforming growth factor-beta (TGF-β). Beyond structural roles, mesangial cells contribute to glomerular development by interacting with podocytes and endothelial cells to form a functional filtration unit. Mesangial cells are also involved in immune responses, expressing class II (MHC-II) and co-stimulatory molecules like CD40 and when activated, allowing them to process antigens and activate CD4+ T cells, which promotes Th1 differentiation and proinflammatory secretion (e.g., IL-6, IL-12). In pathological contexts, such as glomerulonephritides including and , mesangial cell proliferation and matrix expansion lead to sclerosis and impaired filtration, exacerbating kidney injury through inflammatory and fibrotic processes.

Anatomy

Location and Types

Mesangial cells are specialized located within the of the , constituting approximately 30–40% of the total glomerular cellularity. These cells are integral to the mesangium, a supportive structure in the , and are classified into two main types based on their anatomical position: intraglomerular and extraglomerular mesangial cells. Intraglomerular mesangial cells reside between the loops of the glomerular tuft, where they occupy the central mesangial region and contribute to the overall architecture of the filtration unit. These cells extend slender cellular processes that anchor directly to the (GBM), facilitating their spatial organization and interaction with surrounding glomerular components. Extraglomerular mesangial cells, also referred to as Goormaghtigh cells or lacis cells, are situated at the vascular pole of the , specifically in the region between the afferent and within the . These cells form connections to the cells of the adjacent arterioles via gap junctions, enabling coordinated signaling in the renal vascular network.

Morphology and Ultrastructure

Mesangial cells exhibit an irregular, stellate morphology characterized by a central cell body and multiple elongated cytoplasmic processes that extend toward the glomerular basement membrane (GBM) and make contact with adjacent mesangial cells and endothelial cells. These processes extend between and support the glomerular capillaries, integrating the cells into the structural framework of the mesangium. Ultrastructurally, the cells display a prominent rough endoplasmic reticulum, indicative of active protein synthesis, along with lysosomes that contribute to their intracellular processing capabilities. Within the intraglomerular mesangial population, the majority exhibit a contractile , while up to 15% display a /macrophage-like phagocytic derived from precursors. The contractile cells contain bundles of filaments, , and associated proteins such as , organized into a network that traverses the cytoplasmic extensions. Intermediate filaments, including , provide additional cytoskeletal support, with desmin expression also observed in these cells. Connections between mesangial cells and the overlying often occur through fenestrated regions, facilitating close apposition without direct membrane fusion. The mesangial matrix surrounding these cells consists primarily of , , , and proteoglycans such as and agrin, forming a specialized non-cellular compartment that embeds and supports the mesangial cells. This matrix integrates with the GBM at paramesangial angles, creating a cohesive structural unit within the .

Development

Embryonic Origin

Mesangial cells originate primarily from the stromal compartment of the metanephric , a derivative of the that forms during early . These stromal progenitors differentiate into mesangial cells, which exhibit characteristics of and vascular smooth muscle-like cells, providing to the developing glomerular capillaries. Some studies suggest a minor contribution from neural crest-derived stromal cells that integrate into the renal , though the predominant remains mesenchymal. During early nephrogenesis, mesangial cell precursors express key markers such as , which is essential for their recruitment and differentiation, and alpha smooth muscle actin (α-SMA), indicative of their contractile properties. PDGFRβ is detected in perivascular mesenchymal cells surrounding nascent glomerular structures, while α-SMA expression emerges in maturing mesangial cells within the glomerular tuft. These precursors migrate into the developing around embryonic day 13-15 in mice, guided by chemotactic signals including PDGF-B from endothelial cells and (VEGFA). This migration is critical for populating the S-shaped body stage of glomerular formation. Evidence from genetic studies supports this process; PDGF-B null mice exhibit a complete absence of mesangial cells, resulting in disorganized glomerular capillaries and formation of aneurysms due to failed recruitment of PDGFRβ-positive progenitors.

Role in Glomerular Formation

During glomerular development, podocytes, derived from visceral epithelial precursors, secrete (VEGF) to recruit endothelial precursors into the vascular cleft of the S-shaped body, initiating the formation of primitive vascular loops. These endothelial cells then produce B (PDGF-B), which acts in a paracrine manner on PDGF receptor β (PDGFRβ)-expressing mesangial progenitors—originating from perivascular cells surrounding the afferent and efferent arterioles—to recruit and direct their migration into the developing glomerular tuft. This sequential recruitment establishes the foundational cellular architecture, with mesangial cells invading the cleft shortly after endothelial entry, typically around the comma- and S-shaped stages in mammalian models. Mesangial cells play a pivotal role in branching by attaching to endothelial cells and exerting contractile forces that promote capillary looping and , transforming the initial linear vascular structures into a complex, lobulated tuft. Through these interactions, mesangial cells stabilize the nascent against emerging hemodynamic pressures from blood flow, preventing collapse and ensuring structural integrity as the tuft expands. Additionally, mesangial cells engage in with endothelial cells, including the secretion of VEGF isoforms to support endothelial differentiation and formation, while coordinating with podocytes via shared matrix components and growth factors to assemble the barrier. In human fetal kidney development, mesangial cell invasion precedes full maturation, occurring prominently from the 8th to 12th gestational weeks as part of early nephrogenesis, with the glomerular tuft achieving a mature vascular configuration by approximately 20 weeks. This timeline aligns with the transition from capillary loop stages to mature glomeruli, where mesangial cells integrate into the central mesangium to support ongoing tuft refinement.

Function

Structural Support and Matrix Production

Mesangial cells contribute to the structural integrity of the by providing tensile support to the capillary loops, which helps prevent their under the high intraglomerular s encountered during . The glomerular capillary hydrostatic typically reaches approximately 60 mm , generating significant biomechanical stress on the delicate capillary walls. Through their extensions and the intervening mesangial matrix, these cells anchor and stabilize the glomerular tuft, forming a biomechanical unit with the that distributes wall tension and maintains capillary patency. A key aspect of this support involves the synthesis and maintenance of the mesangial , a specialized that occupies roughly 10% of the glomerular volume in healthy kidneys and serves as the primary scaffold between mesangial cells and capillaries. Mesangial cells produce a balanced array of components, including (predominantly α1·α1·α2 heterotrimers), , and nidogen (also known as entactin), which assemble into a network that provides both rigidity and flexibility to withstand forces. This composition differs slightly from the but complements it to ensure overall glomerular architecture. forms the structural backbone, while and nidogen facilitate adhesion and cross-linking among matrix elements and cell surfaces. Mesangial cells also regulate the turnover of this to preserve , primarily through the of matrix metalloproteinases (MMPs) such as MMP-2 and MMP-9, which degrade proteins, and their endogenous inhibitors, tissue inhibitors of metalloproteinases (TIMPs) like TIMP-1 and TIMP-2. This balanced enzymatic activity allows for dynamic remodeling in response to physiological demands, preventing excessive accumulation or degradation that could compromise glomerular structure. The secretion of these proteases and inhibitors by mesangial cells enables precise control over matrix degradation, ensuring long-term stability of the glomerular tuft. In conditions of hemodynamic stress, such as systemic , mesangial cells respond by increasing matrix deposition to reinforce the glomerular structure against elevated pressures. This adaptive process involves upregulated synthesis of collagen IV and , leading to mesangial expansion that helps mitigate capillary wall strain from sustained high glomerular . Such responses highlight the cells' role in buffering mechanical overload, though chronic activation can contribute to pathological changes if unresolved.

Contractile Regulation of Glomerular Blood Flow

Mesangial cells play a critical role in dynamically regulating glomerular blood flow and through their contractile properties, acting as smooth muscle-like that modulate the glomerular surface area available for filtration. of these cells reduces the coefficient (K_f) by decreasing lumen diameter and surface area, thereby attenuating (GFR) without altering systemic . This ensures fine-tuned control of renal in response to physiological demands. Contraction is primarily mediated by vasoactive agonists binding to G-protein-coupled receptors on mesangial cells, triggering intracellular pathways. Angiotensin II, endothelin-1, and are key mediators; for instance, angiotensin II activates , leading to inositol (IP_3)-induced release of Ca²⁺ from intracellular stores, which activates voltage-gated Ca²⁺ channels and sustains contraction via actin- interactions. Similarly, endothelin-1 and elicit comparable Ca²⁺ mobilization and membrane through nonselective cation and chloride channels, resulting in reduced surface area. These processes involve cytoskeletal elements such as and filaments, enabling the cells to exert mechanical force on adjacent capillaries. Relaxation of mesangial cells counteracts contraction to increase K_f and GFR, primarily through cyclic GMP (cGMP)-dependent pathways activated by vasodilatory signals. (ANP) binds to natriuretic peptide receptors, elevating cGMP levels and activating G, which hyperpolarizes the via large-conductance Ca²⁺-activated K⁺ (BK_Ca) channels, thereby inhibiting Ca²⁺ influx and promoting relaxation. (NO), produced locally by endothelial or mesangial cells, similarly stimulates soluble to raise cGMP, enhancing BK_Ca activity and reducing contractile tone to expand the filtration surface. These mechanisms provide a loop to balance hemodynamic forces within the . Mesangial cells integrate with (TGF) to maintain GFR homeostasis, particularly through extraglomerular mesangial cells positioned at the . These cells sense signals from the , such as increased NaCl delivery, and transmit them via gap junctions or paracrine factors to adjust afferent tone, modulating glomerular flow in concert with intraglomerular mesangial contraction. This coordination allows TGF to fine-tune by altering both and dynamics. Quantitatively, mesangial contractions can alter GFR by 20-30% independently of changes in glomerular , as demonstrated in studies where agonist-induced responses reduced single-nephron GFR without systemic hemodynamic shifts. For example, angiotensin II infusion in intact glomeruli decreases GFR by up to 61% in controls, with mesangial tone contributing substantially to this effect. Such modulation underscores the cells' role in preventing glomerular hyperfiltration or hypofiltration under varying physiological conditions.

Phagocytosis and Macromolecule Clearance

Mesangial cells serve as key within the , engulfing apoptotic endothelial and debris through receptor-mediated mechanisms involving such as α_vβ_3 and thrombospondin, independent of CD36. This process facilitates the resolution of by removing cellular remnants without triggering excessive immune responses, as demonstrated in models of self-limited where neighboring mesangial cells apoptotic peers. Additionally, mesangial cells employ and receptor-dependent to internalize aggregated proteins and lipids, utilizing coated pits that direct particles to endosomes and subsequently to phagolysosomes for degradation. In their role as , mesangial cells clear macromolecules such as IgG-containing immune complexes via Fcγ receptors, preventing their accumulation and subsequent inflammatory cascade in the glomerular mesangium. Similarly, they internalize oxidized low-density lipoproteins (oxLDL) through receptors, mitigating potential and lipid-mediated glomerular injury under physiological conditions. These clearance functions are essential for maintaining glomerular , with uptake pathways like those involving β1,4-galactosyltransferase 1 specifically handling IgA1 complexes. Certain mesangial cell populations exhibit monocyte-like characteristics, acquiring a phenotype upon stimulation, which enhances their phagocytic capacity. These monocyte-like mesangial cells manage the engulfment of debris and macromolecules, and upon , they secrete pro-inflammatory cytokines including interleukin-6 (IL-6) to coordinate local immune responses. The phagocytic capacity of mesangial cells is limited, and excessive macromolecular load, such as from persistent immune complexes, can overwhelm lysosomal processing, leading to phagolysosomal destabilization and cellular injury. This overload disrupts lysosomal , impairing degradation and contributing to mesangial dysfunction, as observed when complement induces lysosomal permeabilization. Lysosomal organelles, briefly referenced here, are central to this degradative pathway but can become sites of under stress.

Pathophysiology

Activation and Proliferation in Disease

Mesangial cells undergo in response to various pathological stimuli, including , cytokines such as transforming growth factor-β (TGF-β), and mechanical stretch, which collectively induce phenotypic changes toward a , myofibroblast-like state characterized by increased expression of α-actin (α-SMA). promotes this by altering profiles, including upregulation of actin-regulatory proteins through and cytoskeletal disassembly, while TGF-β enhances and bioactivity in mesangial cells, amplifying the response. Mechanical stretch, often resulting from altered glomerular , triggers signaling cascades like MAP kinase , which is further potentiated under high-glucose conditions, leading to inflammatory and loss of contractile properties. This enables mesangial cells to adopt reparative but potentially maladaptive roles in glomerular injury. Proliferation of mesangial cells, or , is a key response to glomerular injury, primarily mediated by (PDGF) and (EGF) signaling pathways, which drive and increase cell numbers to address damage. PDGF, released by injured mesangial cells and platelets, acts as a potent , with upregulated PDGF A and B chains and receptor expression observed in proliferative glomerulonephritis models, peaking during active injury phases. EGF-family ligands similarly contribute by modulating growth and matrix responses, ensuring coordinated expansion in immune-mediated glomerular insults. This proliferative response, while initially protective, can exacerbate sclerosis if unchecked. In contrast to , mesangial cell —characterized by increased cell size without —predominates in metabolic stresses like in , often linked to arrest mediated by proteins such as p27Kip1 and TGF-β signaling. This contributes to early glomerular expansion without , distinguishing it from the DNA synthesis-driven seen in immune-mediated damages like proliferative . Recent single-cell sequencing studies have elucidated mesangial-specific gene programs in early glomerular disease, revealing upregulated genes (e.g., COL1A1) and components in activated mesangial clusters, highlighting their role in initiating fibrotic pathways before overt .

Role in Extracellular Matrix Expansion

In pathological states, mesangial cells exhibit dysregulated () dynamics, characterized by an imbalance favoring synthesis over degradation, leading to pathological matrix accumulation. This involves upregulation of key components such as and collagens I and III, driven by stimuli like high glucose environments. Concurrently, degradation is impaired due to downregulation of matrix metalloproteinases (MMPs), particularly MMP-2 and MMP-9, which normally break down proteins. A central underlying this is the induction of genes by transforming growth factor-β1 (TGF-β1), which activates signaling pathways promoting transcription of , I, and III in mesangial cells. TGF-β1-mediated effects result in progressive mesangial that can occupy 30% or more of the glomerular tuft volume in advanced disease stages, markedly altering glomerular architecture. The consequences of this ECM expansion include mechanical compression of glomerular capillaries, which reduces the available filtration surface area and impairs (GFR). This compression also contributes to local ischemia by restricting blood flow within the glomerular tuft, exacerbating renal dysfunction. Recent findings highlight the role of microRNA-21 (miR-21) upregulation in promoting deposition through inhibition of in mesangial cells. Under high-glucose conditions, elevated miR-21 suppresses PTEN expression, activating the Akt/ pathway and thereby reducing autophagic degradation of cellular components, which indirectly enhances matrix accumulation. Emerging research as of 2025 has identified additional molecular regulators, including the GABP, which promotes mesangial cell and renal in via epigenetic modifications, and glycine decarboxylase (GLDC), which boosts mesangial in by enhancing . These insights highlight evolving metabolic and transcriptional pathways in mesangial .

Clinical Significance

Diabetic Kidney Disease

In diabetic kidney disease (DKD), chronic profoundly impacts mesangial cells, primarily through the formation of (AGEs), which accumulate and induce by disrupting cellular and elevating (ROS) levels. This oxidative milieu upregulates transforming growth factor-beta (TGF-β), a key profibrotic that stimulates mesangial cell , , and excessive production of components such as IV and , leading to mesangial expansion. The hallmark pathological feature, Kimmelstiel-Wilson nodules, arises from this progressive matrix accumulation and mesangial cell , forming acellular, PAS-positive sclerotic nodules that compress glomerular capillaries and contribute to nodular . Mesangial cell hypercontractility further exacerbates glomerular in DKD, as and mechanical strain from elevated intraglomerular pressure enhance contractility via vasoactive mediators like angiotensin II, reducing capillary surface area and intensifying hyperfiltration injury. This contractile dysfunction, coupled with ROS-mediated signaling, perpetuates a cycle of injury that impairs glomerular and promotes . As DKD advances to sclerosis, mesangial cells undergo driven by sustained and pathways such as PTEN/AKT dysregulation, leading to cell loss, irreversible , and glomerular obsolescence; in patients with , in the cumulative risk of progression to end-stage renal disease (ESRD) reaches approximately 50% within 10 years, while in the risk is lower, ranging from 3-11%. Recent therapeutic advances include sodium-glucose cotransporter 2 (SGLT2) inhibitors like empagliflozin, which express in mesangial cells and mitigate by blocking local , reducing ROS and , and attenuating TGF-β-driven matrix expansion, thereby slowing DKD progression independently of systemic glycemic control.

IgA Nephropathy and Other Glomerular Diseases

In , the most common primary glomerulopathy worldwide, deposition of galactose-deficient IgA1-containing immune complexes in the mesangium activates mesangial cells, leading to their proliferation, release, and excessive production. This mesangial expansion impairs glomerular filtration and contributes to the hallmark clinical features of recurrent gross and persistent , often progressing to if untreated. A phase 3 published in 2025 demonstrated that atacicept, a dual inhibitor of B-cell activating factors, significantly reduced and in patients with by suppressing pathogenic IgA production and thereby attenuating mesangial inflammation. Mesangial cells are also central to the pathology of other immune-mediated glomerular diseases. In , particularly class II mesangial proliferative forms, immune complex deposition induces mesangial hypercellularity and matrix accumulation, exacerbating and renal . involves complement activation within the mesangium, often via the alternative pathway, which triggers mesangial proliferation and subendothelial deposits, leading to glomerular capillary wall thickening and hypocomplementemia. In , maternal autoantibodies against the II type 1 receptor (AT1-AA) bind to mesangial cells, stimulating secretion of interleukin-6 (IL-6) and (PAI-1), which promote glomerular endotheliosis and systemic . Across these disorders, a shared involves overload of mesangial phagocytic capacity due to excessive immune complex uptake, resulting in , dysregulation, and direct cell injury that amplifies glomerular damage. Additionally, extraglomerular mesangial cells, which interface with components, contribute to renin release dysregulation during , potentially exacerbating and renal hypoperfusion in these conditions. Mesangial alterations, such as proliferation and matrix expansion, are observed in approximately 20-30% of primary glomerulopathies based on registries, underscoring their prevalence in non-diabetic glomerular injury.