NLRC4

NLRC4 (NLR family CARD domain-containing protein 4) is a protein that in humans is encoded by the NLRC4 gene. It is involved in the immune system and plays a crucial role in the body's defense against pathogens.

Function

NLRC4 is a member of the NLR family of proteins, which are involved in the regulation of the immune response. These proteins are characterized by a tripartite domain architecture, including a central nucleotide-binding domain (NBD), an N-terminal effector-binding domain, and a C-terminal leucine-rich repeat (LRR) domain. The LRR domain is thought to be involved in the recognition of pathogen-associated molecular patterns (PAMPs).

NLRC4 is known to form an inflammasome, a multiprotein complex that activates inflammatory responses. The NLRC4 inflammasome is activated in response to certain bacterial pathogens, leading to the activation of the pro-inflammatory cytokines IL-1β and IL-18.

Clinical significance

Mutations in the NLRC4 gene have been associated with a number of autoinflammatory diseases, including familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and neonatal-onset multisystem inflammatory disease. These diseases are characterized by recurrent episodes of fever, skin rash, and joint pain.

In addition, mutations in NLRC4 have been implicated in the development of macrophage activation syndrome, a severe, life-threatening illness characterized by excessive activation and proliferation of certain immune cells.

See also

• NLRP3
• Inflammasome
• Autoinflammatory disease
• Macrophage activation syndrome

External links

• NLRC4 at NCBI
• NLRC4 at UniProt

NLRC4 Resources
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