The folate metabolic pathways. Blue ellipses, enzymes; green rectangles, metabolites; written in red, names of genes selected for genotype analysis; underlined, genes that showed a significant association with certain types of CHD (contruncal, septal, and LVOTO). ABCB1, P-glycoprotein; ABCC3, multidrug resistant protein 3; SLC19A1; solute-carrier family 19; FPGS, folypolyglutamyl synthase; FOLglu, polyglutamylated folic acid; DHF, dihydrofolate; DHFR, dihydrofolate reductase; THF, tetrahydrofolate; MTHFD1, trifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolase/synthase; 10-CHO-THF, 10-formyl tetrahydrofolate; 5,10 = CH-THF, methenyl tetrahydrofolate; 5,10-CH2 = THF, methylene tetrahydrofolate; 5-CH3-THF, 5-methyl tetrahydrofolate; MTR, 5-methyl tetrahydrofolate-homocysteine methyltransferase; MTRR, 5-methyl tetrahydrofolate-homocysteine methyltransferase reductase; MAT2A, methionine adenosyltransferase II alpha; SAM, S-adenosylmethionine; SAH, S-adenosylhomocysteine; GNMT, glycine N-methyltransferase; DNMT3B, DNA (cytosine-5-)-methyltransferase 3 beta; ACHYL1, S-adenosylhomocysteine hydrolase-like 1; BHMT, betaine-homocysteine S-methyltransferase; diMeGly, dimethylglycine; Me, methyl; LVOTO, left ventricular outflow tract obstruction; CHD, congenital heart defect.