What is IFIH1 gene?

The IFIH1 gene provides instructions for making the MDA5 protein, which plays an important role in innate immunity, the body’s early, nonspecific response to foreign invaders (pathogens) such as viruses and bacteria.

What is interferon gene?

Interferons (IFNs, /ˌɪntərˈfɪərɒn/) are a group of signaling proteins made and released by host cells in response to the presence of several viruses. In a typical scenario, a virus-infected cell will release interferons causing nearby cells to heighten their anti-viral defenses.

Is MDA5 a gene?

MDA5 (melanoma differentiation-associated protein 5) is a RIG-I-like receptor dsRNA helicase enzyme that is encoded by the IFIH1 gene in humans. MDA5 is part of the RIG-I-like receptor (RLR) family, which also includes RIG-I and LGP2, and functions as a pattern recognition receptor capable of detecting viruses.

Does chicken contain interferon?

The three types of IFNs display distinct expression patterns and have each key role in innate and adaptive immunity. Interestingly, the first identified IFN was chicken interferon, originally defined as a factor that interferes with influenza virus replication in chicken chorioallantoic membrane [2].

Are interferons inflammatory?

The innate immune response is involved in various inflammatory processes and has a particularly important role in bacterial and viral infections. Interferons (IFNs) and inflammatory cytokines are crucial molecules in this process, influencing cellular, tissue, and global physiological functions.

Where does interferon come from?

Type I interferon (IFN-alpha and IFN-beta) is secreted by virus-infected cells while type II, immune or gamma interferon (IFN-gamma) is mainly secreted by T cells, natural killer (NK) cells and macrophages.

What triggers interferon production?

Interferon is secreted by cells in response to stimulation by a virus or other foreign substance, but it does not directly inhibit the virus’s multiplication. Rather, it stimulates the infected cells and those nearby to produce proteins that prevent the virus from replicating within them.

What does MDA5 bind to?

double-stranded RNA
IFIH1, also known as MDA5, is a cytoplasmic receptor that binds double-stranded RNA. Activation of IFIH1 is part of the inflammatory response to viral RNA replication, again resulting in the induction of type I IFN.

What is MDA5 autoimmune disease?

Anti-MDA5 dermatomyositis is a rare systemic autoimmune disease, historically described in Japanese patients with clinically amyopathic dermatomyositis and life-threatening rapidly progressive interstitial lung disease.

Are interferons inflammatory cytokines?

Interferons (IFNs) and pro- and anti-inflammatory cytokines are crucial for appropriate response to pathogens, damaged cells, or irritants in inflammatory response. The inflammasom is multiprotein complex, which initiates cleavage of pro-inflammatory cytokines IL-1β and IL-18 into active forms.

Is MDA5 genetic?

Inheritance. The inheritance pattern of MDA5 deficiency is unclear. In some cases, the condition seems to follow an autosomal recessive pattern , which means both copies of the gene in each cell have mutations.

What are the symptoms of MDA5?

Skin ulcerations associated with anti-MDA5 DM usually manifest as deep painful ulcers localized over Gottron’s papule, involving the digital pulp and nail folds, or over Gottron’s sign on the knees, elbows, or both.

What causes MDA5 antibody?

The MDA5 antibody, also known as an anti-CADM-140 antibody, has especially been associated with CADM [6], and has been used to detect dermatopulmonary syndrome in patients who have undergone allogeneic hematopoietic stem cell transplantation [9] or in patients with DM [10, 11].

What is dermatomyositis MDA5?

Clinical characteristics of MDA5-positive dermatomyositis. DM is a rare autoimmune disease characterized by skin involvement, striate muscle inflammation which may include the upper oesophagus and possible damage of internal organs frequently marked by the presence of autoantibodies [23].

How is MDA5 activated?

recently showed that MDA5 can be activated by long dsRNA from the genome of reoviruses (ReoVs) or made by annealing sense and antisense strands of in vitro transcribed RNA (10). Therefore, it has come to be believed that the physiological agonist for MDA5 is simply long molecules of dsRNA.

Where are NLRs found?

NLRs can cooperate with toll-like receptors (TLRs) and regulate inflammatory and apoptotic response. They are found in lymphocytes, macrophages, dendritic cells and also in non-immune cells, for example in epithelium. NLRs are highly conserved through evolution.

How serious is dermatomyositis?

Dermatomyositis and polymyositis are serious diseases with a disease‐related mortality of at least 10%. In the long term, myositis has a major effect on perceived disability and quality of life, despite the regained muscle strength.

Does dermatomyositis shorten your life?

Conclusions: High mortality occurred in the first year, and the survival rate decreased continually up to 9 years. The main prognostic factor for death is old age, but dermatomyositis and polymyositis must be considered separately.

What are plant NLRs?

Plants require a complex immune system to defend themselves against a wide range of pathogens which threaten their growth and development. The nucleotide-binding leucine-rich repeat proteins (NLRs) are immune sensors that recognize effectors delivered by pathogens.

What is the main outcome of pathogen recognition by CLRs?

Unlike the predominantly protective responses that CLRs mediate to other pathogens, viral recognition by CLRs tends to favour transmission, infection and inflammation.