What is MOGS-CDG?

MOGS-CDG is a form of congenital disorders of N-linked glycosylation characterized by generalized hypotonia, craniofacial dysmorphism (prominent occiput, short palpebral fissures, long eyelashes, broad nose, high arched palate , retrognathia), hypoplastic genitalia, seizures, feeding difficulties, hypoventilation, severe hypogammaglobulinemia with generalized edema, and increased resistance to particular viral infections (particularly to enveloped viruses). The disease is caused by loss-of-function mutations in the gene MOGS (2p13.1).

Other condition names

  • CDG syndrome type IIb
  • CDG-IIb
  • CDG2B
  • Carbohydrate deficient glycoprotein syndrome type IIb
  • Congenital disorder of glycosylation type 2b
  • Congenital disorder of glycosylation type IIb
  • Glucosidase 1 deficiency

Inheritance type

Autosomal recessive


  • Worldwide: <1 in 1,000 000

Age of Onset

  • Infancy
  • Neonatal
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