One study showed decreased NALP1
and CASP1 gene expression in septic shock patients compared with critically ill patients and healthy volunteers, supporting the conclusion that the changes in the inflammasome are part of the monocyte deactivation process that occurs in septic patients (31), while another study reported increased CASP1, IL-1[beta], and IL-18 expression in septic patients with acute respiratory distress syndrome (ARDS) compared with SIRS, supporting that the inflammasome pathway and its downstream cytokines play critical roles in ARDS development (32).
Bruey et al., "Reconstituted NALP1 inflammasome reveals two-step mechanism of caspase-1 activation," Molecular Cell, vol.
Luciano et al., "Bcl-2 and Bcl-XL regulate proinflammatory caspase-1 activation by interaction with NALP1," Cell, vol.
There have been seven established NLRs that form an inflammasome complex: NLRP1 (NALP1
), NLRP3 (NALP3 or cryopyrin), NLRP6, NLRP12, NLRC4 (with caspase recruitment domain or IPAF), AIM2 (absent in melanoma-2), and RIG-1 (retinoic acid inducible gene-1); however, the NLRP3 inflammasome is the best characterized in relation with renal diseases .
Various susceptibility loci are AIS1 (chr1), AIS2 (chr7), AIS3 (chr8), SLEV1 (chr17), VIT 1 GENE on chromosome 2p16 and gene NALP1 on chromosome 17p13.
NALP1 in vitiligo associated multiple autoimmune disease.
NLRP1 (also known as CARD7, NAC, DEFCAP, and NALP1) was the first identified NLR-family protein [5, 6] and differs from other NLR-family proteins in that its C-terminal region contains a nucleotide-binding domain (NBD).
Sun et al., "DAC can restore expression of NALP1 to suppress tumor growth in colon cancer," Cell Death & Disease, vol.
Everett et al., "Inflammasome components NALP1
and 3 show distinct but separate expression profiles in human tissues suggesting a site-specific role in the inflammatory response," Journal of Histochemistry and Cytochemistry, vol.
El NLRP1 tambien conocido como NALP1
, es el sensor de las senales de activacion de la respuesta inmune innata y esta expresado en muchos tipos de celulas inmunocompetentes en particular las celulas de Langerhans de la piel (35).
For example, mutations in NALP1
have been shown to be associated with autoimmune vitiligo in conjunction with a number of different systemic autoimmune diseases.
An increased frequency of mutated BclI genotype in the glucocorticoid receptor gene NR3C1 (nuclear receptor subfamily 3, group C, member 1) was described in responsive IBD patients compared with nonresponders but a mutation in the NALP1
gene was significantly more frequent in resistant patients (35).