It might be possible that c.-1973T >C influences the genesis of liver disease in childhood, in line with a recently published report implicating that a variant of the endoplasmic reticulum mannosidase I (ERManI) gene is associated with an early onset of end-stage liver disease in patients with homozygous (Pi ZZ) A1AT deficiency[14]. Here, MAN1B1 is linked to hyperinsulinemic hypoglycemia, familial, 4.