Table 1.

Summary of IAP gene mutation or ablation phenotypes in human and mouse

Protein	Mutation in human	Knockout in mice	Ref
Survivin	NR*	Lethal at early embryonic stage. Loss in thymocytes causes mitotic defects.	(Okada et al., 2004; Uren et al., 2000)
XIAP	Loss of expression causes an X-linked lymphoproliferative syndrome with low numbers of NKT cells.	Viable. Delayed lobuloalveolar development in mammary gland.	(Rigaud et al., 2006; Harlin et al., 2001; Olayioye et al., 2005)
c-IAP1	Deletion of c-IAP1/c-IAP2 associated with increased activation of the non-canonical NF-κB pathway in multiple myeloma patients.	Viable. Loss of c-IAP1 causes elevated levels of c-IAP2 expression.	(Conze et al., 2005; Keats et al., 2007)
c-IAP2	c-IAP2/MALT1 fusion protein, generated by chromosomal translocation associated with MALT lymphoma, activates NF-κB. Deletion of c-IAP1/c-IAP2 associated with increased activation of the non-canonical NF-κB pathway in multiple myeloma patients.	Viable. Mice are resistant to LPS-induced shock. Protects macrophages from LPS-induced death.	(Conte et al., 2006; Zhou et al., 2005; Keats et al., 2007)
NAIP	NR	Multiple alleles of NAIP are present in different strains of mice. Mice lacking NAIP5 are susceptible to Legionella pneumophila infection.	(Wright et al., 2003)
BRUCE	NR	Embryonic lethal. Apollon-deficient MEFs are sensitive to SMAC-induced apoptosis. Knockout of UBC domain alone causes apoptosis in placenta and yolk sac.	(Hao et al., 2004; Ren et al., 2005)

^*No mutation reported