Table 2.

Candidate gene modifiers of clinical phenotypes in cystic fibrosis

Gene	Lung function (FEV1)	Ps. a. acquisition	Meconium ileus	CF-related diabetes	CF liver disease	References
MBL2	REPLICATEDa	REPLICATED				Gabolde et al. 1999; Garred et al. 1999; Davies et al. 2004; Yarden et al. 2004; Trevisiol et al. 2005; Dorfman et al. 2008; McDougal et al. 2010
EDNRA	REPLICATED					Darrah et al. 2010
TGF-β1	REPLICATED	Negativec			Probably negatived	Arkwright et al. 2000, 2003; Drumm et al. 2005; Brazova et al., 2006; Bremer et al. 2008; Corvol et al. 2008; Dorfman et al. 2008; Bartlett et al. 2009; Faria et al. 2009
IFRD1	Replicatedb					Gu et al. 2009
IL8	Replicated					Hillian et al. 2008
MSRA			REPLICATED			Henderson et al. 2012
ADIPOR2			Replicated			Dorfman et al. 2009
TCF7L2				REPLICATED		Blackman et al. 2009a
SERPINA1	Negative	Probably negative			Replicated	Arkwright et al. 2000, 2003; Drumm et al. 2005; Brazova et al. 2006; Bremer et al. 2008; Corvol et al. 2008 Dorfman et al. 2008; Bartlett et al. 2009; Faria et al. 2009

^aREPLICATED: seen in ≥3 populations with total ≥1000 individuals.

^bReplicated: seen in ≥2 populations with total ≥500 individuals.

^cNegative: negative in ≥3 populations with total ≥1000 individuals.

^dProbably negative: negative in ≥2 populations with total ≥500 individuals.