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Tumor necrosis factor

Tumor necrosis factor (TNF), formerly known as TNF-α, is a chemical messenger produced by the immune system that induces inflammation. TNF is produced primarily by activated macrophages, and induces inflammation by binding to its receptors on other cells. It is a member of the tumor necrosis factor superfamily, a family of transmembrane proteins that are cytokines, chemical messengers of the immune system. Excessive production of TNF plays a critical role in several inflammatory diseases, and TNF-blocking drugs are often employed to treat these diseases.

History
In the 1890s, William Coley observed that acute infections could cause tumor regression, leading to his usage of bacterial toxins as a cancer treatment. In 1944, endotoxin was isolated from Coley's bacterial toxins as the substance responsible for the anticancer effect. In particular, endotoxin could cause tumor regression when injected into mice with experimentally induced cancers. In 1975, Carswell et al. discovered that endotoxin did not directly cause tumor regression, but instead induced macrophages to secrete a substance that causes tumors to hemorrhage and necrotize, termed "tumor necrosis factor." == Evolution ==
Evolution
The TNF and lymphotoxin-α genes are believed to be descended from a common ancestor gene that developed early in vertebrate evolution, before the Agnatha and Gnathostomata split. This ancestor gene was dropped from the Agnatha ancestor but persisted in the Gnathostomata ancestor. During the evolution of gnathostomes, this ancestor gene was duplicated into the TNF and lymphotoxin-α genes. Thus, while the ancestor gene is found across a variety of gnathostome species, only a subset of gnathostome species contain a TNF gene. Some fish species, such as Danio, have been found to contain duplicates of the TNF gene. The TNF proximal promoter region is also highly conserved among mammals, and nearly identical among higher primates. The similarity of the TNF gene among fish is lower, ranging from 226 to 256 amino acids. Like mammalian TNF, the fish TNF gene has been shown to be stimulated in macrophages by antigens. All TNF genes have a highly conserved C-terminal module known as the TNF homology domain, due to its important role in binding TNF to its receptors. == Gene ==
Gene
Location The human TNF gene is mapped to chromosome 6p21.3, residing in the class III region of the major histocompatibility complex, where many immune system genes are contained. The class III region is sandwiched between the HLA-DR locus on the centromeric side, and the HLA-B locus on the telomeric side. The TNF gene is 250 kilobases away from the HLA-B locus, and 850 kilobases away from the HLA-DR locus. The TNF gene is located 1,100 kilobases downstream of the lymphotoxin-α gene. Expression . The composition of the enhanceosome depends on ambient factors within the cell, particularly nuclear factor of activated T-cells (NFAT).|alt=Diagram of human TNF exons and introns The transcribed region contains 4 exons separated by 3 introns, for a total of 2,762 base pairs in the primary transcript and 1,669 base pairs in the mRNA. The mRNA consists of four regions: the 5′ untranslated region, which is not included in the TNF protein; the transmembrane portion, which is present in transmembrane TNF but not in soluble TNF; the soluble portion; and the 3′ untranslated region. More than 80% of the soluble portion is contained in the last exon, while the transmembrane portion is contained in the first two exons. The 3' untranslated region contains an AU-rich element (ARE) that regulates the translation of TNF. In unstimulated macrophages, various proteins bind to the ARE to destabilize TNF mRNA, suppressing the translation of TNF. Upon activation, TNF translation is unsuppressed. == Protein ==
Protein
, whereas individual monomers are inactive. The coexistence of TNF dimers and trimers in dynamic equilibrium suggests that TNF might be a morpheein. Small molecules that stabilize TNF dimers and prevent the assembly of TNF trimers present a potential mechanism for inhibiting TNF. == Function ==
Function
TNF is a central mediator of the body's innate immune response. Additionally, transmembrane TNF (tmTNF) acts as a reverse signaler, triggering a variety of responses in its own cell depending on cell type and stimulant. TNFR1 signaling , tmTNF reverse signalling has been shown to increase NF-κB activity, enhancing cell survival and apoptosis resistance. In natural killer cells, tmTNF reverse signalling increases cytotoxic activity by increasing the expression of perforin, granzyme B, Fas ligand, and TNF. In T cells, the activation of the JNK pathway by tmTNF reverse signalling can lead to cell cycle inhibition and apoptosis. TNF is the principal cytokine for regulating acute inflammation, though many of its functions are shared with other cytokines, especially IL-1. By binding to TNF receptors, TNF can perform functions including stimulating endothelial cells to induce coagulation, which obstructs blood flow to prevent the spread of microbes; stimulating endothelial cells and macrophages to secrete chemokines that attract white blood cells; stimulating the secretion of other cytokines such as IL-1; activating neutrophils and macrophages; stimulating the liver to produce acute phase proteins, such as C-reactive protein; inducing catabolism of muscles and fat to produce energy; and stimulating scar tissue formation, also known as fibrosis. In addition to inducing the secretion of cytokines, TNF itself can be induced by cytokines, enabling a cascade of inflammatory signals. Excessive amounts of TNF can cause septic shock. Central nervous system TNF is expressed in various cells in the central nervous system, including glial cells, microglia, astrocytes, and neurons, and plays a critical role in maintaining homeostasis. Through TNFR1 signalling, TNF can increase the surface expression of AMPA receptors and NDMA receptors in neurons, strengthening synaptic transmission. TNF also decreases the surface expression of GABAA receptors, reducing the activity of inhibitory synapses. TNF can also modulate the release of glutamate, an excitatory neurotransmitter, and S100B, a zinc-binding protein, by astrocytes. The modulation of excitation and inhibition of neurons by TNF indicates that TNF plays a role in synaptic scaling and plasticity. Through TNFR2 signalling, TNF promotes the proliferation and maturation of oligodendrocytes, which produce protective myelin sheaths around nerve cells. On the other hand, TNF becomes cytotoxic to oligodendrocyte progenitor cells when the cells are in contact with astrocytes. == Clinical significance ==
Clinical significance
Autoimmunity Excessive production of TNF plays a key role in the pathology of autoimmune diseases, such as rheumatoid arthritis, inflammatory bowel disease, psoriatic arthritis, psoriasis, and noninfectious uveitis. In these diseases, TNF is erroneously secreted by immune cells in response to environmental factors or genetic mutations. TNF then triggers an inflammatory response, damaging normal tissue. TNF blockers, which prevent TNF from binding to its receptors, are often used to treat these diseases. In some cancers, TNF has been shown to play an inhibitory role, primarily when injected locally, repeatedly, and at high concentrations. Due to TNF's adverse side effects, potential TNF cancer treatments seek to maximize cytotoxicity to tumors while minimizing exposure to the entire body. Some treatments increase cytotoxicity by inhibiting the cell survival pathways of tumors before treatment with TNF. Other treatments localize TNF activity using antibody-TNF fusions, also known as immunocytokines. Local TNF treatment has been shown to induce tumor regression, though they rarely induce complete remission. Body-wide administration of TNF has shown low efficacy and high side effects. Conversely, TNF plays a role in the progression of HIV by inducing apoptosis of T cells in HIV-infected people. TNF blockage has reportedly led to clinical improvement in HIV without worsening the infection, though data is limited. Although TNF blockers showed efficacy in treating sepsis in mice, they showed mixed results in treating sepsis in humans. This is believed to be due to the dual role that TNF plays in the immune system; blocking TNF reduces the severe inflammation that causes sepsis, but also hinders the immune system's ability to resist the infection. It is hypothesized that TNF blockers are more beneficial in cases of severe sepsis, where the probability of death is higher. Despite this connection, TNF blockers are not used to treat liver fibrosis. In clinical trials of alcoholic hepatitis, TNF blockers had no significant effect. Nonalcoholic fatty liver disease TNF plays a key role in nonalcoholic fatty liver disease (NAFLD), in which fat builds up in the liver, leading to injury, inflammation, and scarring. TNF promotes insulin resistance, which promotes fat build up in the liver. As fat builds up in the liver and surrounding adipose tissue, immune cells may infiltrate the expanding tissue and secrete TNF, causing inflammation. Thus, TNF may serve as a causal link between inflammation, insulin resistance, and fat accumulation in the liver. Clinical studies have shown that TNF levels are correlated with the severity of NAFLD, although some studies have shown otherwise. Pharmacological strategies that downregulate TNF have shown favorable effects on NAFLD, while the efficacy of TNF blockers is yet to be evaluated. Muscle wasting Conditions that cause inflammation, such as cancer, can elevate TNF levels, which contributes to muscle wasting. TNF contributes to muscle wasting by activating the NF-κB pathway, which activates the ubiquitin–proteasome pathway to degrade protein, and by inhibiting the activation of satellite cells, which are responsible for protein regeneration. However, TNF blockers have had limited effect on muscle wasting in clinical studies, likely due to the multifactorial nature of muscle wasting. Exercise During exercise, the level of IL-6, a TNF inhibitor, rapidly increases, leading to an anti-inflammatory effect. This is followed by a subsequent increase in the levels of IL-10 and soluble TNF receptors, both of which also inhibit TNF. While moderate exercise does not increase TNF levels, strenuous exercise has been shown to increase TNF levels two-fold, causing a pro-inflammatory effect. However, this proinflammatory effect is outweighed by the anti-inflammatory effect of IL-6, which can increase 50-fold. Regular exercise has been shown to reduce base TNF levels in the long term. Thus, exercise is generally considered to inhibit TNF, which contributes to the overall anti-inflammatory effect of exercise. Neuroinflammation In the central nervous system, TNF is primarily produced by microglia, a type of macrophage, but also by neurons, endothelial cells, and immune cells. Excessive TNF contributes to neuroinflammation by causing excitotoxic neuronal cell death, increasing glutamate levels, activating microglial cells, and disrupting the blood–brain barrier. As a result, TNF is seen to play an important role in central nervous system disorders associated with neuroinflammation, including neurosarcoidosis, multiple sclerosis, Neuro-Behçet's disease. Paradoxically, TNF-blockers can cause demyelination of neurons and worsen multiple sclerosis symptoms. This is believed to be due to the homeostatic role of TNF in the central nervous system, especially on neuron myelination via TNFR2. The selective blockade of TNFR1 has shown positive outcomes in animal models. TRAPS In TNF receptor associated periodic syndrome (TRAPS), genetic mutations in TNFR1 lead to defective binding of TNFR1 to TNF, as well as defective shedding of TNFR1, a mechanism that attenuates TNFR1 signalling. This causes periodic inflammation, though the exact mechanism is unknown. TNF blockers such as etanercept have shown partial efficacy in reducing symptoms, while other TNF blockers such as adalimumab and infliximab have been shown to worsen symptoms. Taste perception Excessive levels of inflammatory cytokines, such as during infection or autoimmunity, have been associated with anorexia and reduced food intake. It is hypothesized that TNF reduces food intake by increasing sensitivity to bitter taste, though the exact mechanisms of this are unknown. == Pharmacology ==
Pharmacology
TNF blockers TNF blockers bind to TNF to prevent it from activating its receptors. Additionally, TNF blockers that bind to tmTNF may induce apoptosis in TNF-expressing cells, eliminating inflammatory immune cells. TNF blockers can be monoclonal antibodies, such as infliximab, while others are decoy fusion proteins, like etanercept. == References ==
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