Immune system diseases
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Immune System Diseases: An Overview
Introduction to the Immune System
The immune system is a complex network of organs, cells, and molecules that work together to defend the body against harmful pathogens. It consists of two main components: the innate and adaptive immune systems. The innate immune system provides the first line of defense and is responsible for immediate responses to infections, while the adaptive immune system offers a more specialized and long-lasting defense1 6.
Immunodeficiency Disorders
Immunodeficiency disorders occur when parts of the immune system are missing or not functioning properly. This can lead to increased susceptibility to infections. Severe combined immunodeficiency (SCID) is a prime example, where a lack of an enzyme causes toxic waste to build up in immune cells, leading to their destruction and a severely compromised immune system5. Another example is DiGeorge syndrome, which results from improper development of the thymus gland, leading to diminished T cell production5.
Autoimmune Diseases
Autoimmune diseases arise when the immune system mistakenly attacks the body's own tissues. This occurs due to a failure in the regulatory mechanisms that maintain self-tolerance. Diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and multiple sclerosis (MS) are examples where the immune system targets its own cells2 3. The innate immune system plays a crucial role in the initiation and progression of these diseases by regulating the activation of the adaptive immune system and mediating tissue damage through pro-inflammatory cytokines2 3 4.
Autoinflammatory Diseases
Autoinflammatory diseases are characterized by recurrent episodes of systemic and organ-specific inflammation. These conditions often involve the innate immune system and can include both monogenic disorders, like periodic fever syndromes, and more complex multifactorial conditions7. Recent advances have highlighted the role of inflammasomes and other intracellular signaling systems in the pathogenesis of these diseases, leading to a shift towards a systems-based classification of innate immune-mediated diseases7.
The Role of the Microbiome
The microbiome, consisting of commensal microbes, plays a critical role in the development and function of the immune system. Imbalances in microbiota-immunity interactions can contribute to the pathogenesis of various immune-mediated disorders. Understanding these interactions is crucial for developing microbiome-targeted therapeutic interventions9.
Neurodegenerative Diseases and Innate Immunity
The innate immune system also plays a significant role in neurodegenerative diseases. Microglia, the key innate immune cells in the brain, are involved in brain development and homeostasis. Impaired microglial function can lead to inflammation, contributing to diseases such as Alzheimer's, Parkinson's, and Huntington's disease8.
Conclusion
The immune system is essential for protecting the body against infections and maintaining homeostasis. However, its dysregulation can lead to a variety of diseases, including immunodeficiency, autoimmune, and autoinflammatory disorders. Advances in understanding the roles of the innate and adaptive immune systems, as well as the microbiome, are paving the way for new therapeutic approaches to treat these complex diseases.
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Most relevant research papers on this topic
An overview of the immune system
The immune system plays a crucial role in host defense by recognising, repelling, and eradicating pathogens and foreign molecules, with underactivity leading to severe infections and tumors, and overactivity leading to allergic and autoimmune diseases.
Highly CitedInnate immunity as the trigger of systemic autoimmune diseases.
Innate immunity plays a crucial role in triggering and perpetuating autoimmune diseases, with its improper activation leading to diseases like rheumatoid arthritis, systemic lupus erythematosus, multiple sclerosis, and inflammatory bowel disease.
Very Rigorous JournalEditorial: Innate Immunity Pathways in Autoimmune Diseases
Innate immunity plays a significant role in the initiation and progression of autoimmune diseases, highlighting the need to integrate innate and adaptive immune responses in understanding autoimmune diseases.
The innate immune system and cell death in autoinflammatory and autoimmune disease.
Recent advances in understanding innate immunity suggest potential for developing new treatments targeting innate immune signaling and programmed cell death pathways in autoimmune diseases.
The Human Immune Response
Human immunity consists of two primary components: innate and adaptive, with innate immunity based on recognition of microbe-associated molecules and adaptive immunity based on distinction between "self" and foreign molecules.
Moving towards a systems-based classification of innate immune-mediated diseases
A systems-based classification of autoinflammatory diseases, considering multiple molecular mechanisms and systems, could improve our understanding and treatment of these complex disorders.
Innate Immunity and Neurodegeneration.
Innate immunity, particularly through microglia, contributes to neurodegeneration by causing inflammation and impairing brain development and maturation.
Highly CitedInteraction between microbiota and immunity in health and disease
Microbiome-immunity interactions play crucial roles in maintaining health and contributing to immune-mediated disorders, with potential for future microbiome-targeted therapeutic interventions.
Rigorous JournalHighly CitedHuman immune system variation
Human immune systems vary between individuals, with symbiotic and pathogenic microbes being the main contributors, and understanding these influences is crucial for defining immune health metrics and predicting disease risk.
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