Table 1.

Mouse models deficient for genes involved in the control of heme homeostasis.

Heme-related gene	Mouse model	Phenotype	References
ALAS2	Alas2−/− mice	Embryonic lethality; Impaired erythropoiesis; iron accumulation in primitive erythroid cells	Nakajima et al., 1999; Harigae et al., 2003
HEMOPEXIN (Hx)	Hx−/− mice	Endothelial dysfunction, oxidative tissue damage, iron tissue iron redistribution following heme overload	Vinchi et al., 2008, 2013
HEME-OXYGENASE 1 (HO-1)	Ho-1−/− mice	Hepatic and renal iron loading; splenic atrophy; macrophage depletion	Poss and Tonegawa, 1997; Kovtunovych et al., 2010
FLVCR1	Flvcr1a/b−/− mice	Embryonic lethality; Impaired erythropoiesis; Reduced growth and skeletal malformations	Keel et al., 2008
	Flvcr1a/b−/− postnatal mice	Macrocytic anemia	Keel et al., 2008
	Flvcr1a−/− mice	Embryonic lethality; Hemorrhages, edema and skeletal malformations	Chiabrando et al., 2012
	Liver conditional Flvcr1a−/− mice (Flvcr1aflox/flox; Alb-Cre)	Hepatic heme and iron loading; reduced heme synthesis and cytochrome P450 expression/activity in hepatocytes	Vinchi et al., 2014
ABCG2	Abcg2−/− mice	Photosensitization, PPIX accumulation	Jonker et al., 2002
HCP1	Hcp1/PCFT−/− mice	Mortality at 3 months; systemic and cerebrospinal folate deficiency; severe macrocytic normochromic anemia; pancytopenia; liver iron accumulation	Salojin et al., 2011