α-Endomannosidase (MANEA), encoded by the MANEA gene on chromosome 6q16.1, is an enzyme that catalyzes the release of glucosyl-mannose oligosaccha-rides by cleaving the α-1,2-mannosidic bond that links them to high-mannose N-glycans.35 Comparative genomic analysis has demonstrated high-sequence conservation in humans, rats, and mice.36 Human MANEA is expressed in a variety of tissues including brain, though levels of MANEA in the brain are much lower than, for example, in the liver or kidney.36 The role of MANEA is poorly understood but has been hypothesized to be involved in the quality control of N-glycosylation,37 providing cells with the ability to recover and properly mature glucosylated structures that have bypassed glucosidase trimming by glucosidases I and II in the endoplasmic reticulum.36
