• Golgi resident mannosidase that cleaves the final two α1,3-Man and α1,6-Man during N-glycan processing.
• Man2α2 and Man2a1 act as isozymes, with similar α1,3/6-Man specificity.
• Absence of both is necessary for depeletion of complex N-glycans in mice, suggesting functional redundancy.
• Mice lacking MAN2A1 exhibit many of the features of humans lacking this enzyme activity including significant dyserythropoiesis, anemia, splenomegaly, and immature erythrocytes or reticulocytes in the peripheral blood, but no neurologic or behavioral symptoms, no reproductive problems, and no major histological findings in most tissues.
• Man2α2-deficient male mice are largely inferile, but females are normal.
• Swainsonine is a α-Mannosidase inhibitor.