Glycoproteinosis Explained

Glycoproteinosis are lysosomal storage diseases^[1] affecting glycoproteins, resulting from defects in lysosomal function. The term is sometimes reserved for conditions involving degradation of glycoproteins.^[2]

Types

• (E77.0) Defects in post-translational modification of lysosomal enzymes
  • Mucolipidosis II (I-cell disease)
  • Mucolipidosis III (pseudo-Hurler polydystrophy)
• (E77.1) Defects in glycoprotein degradation
  • Aspartylglucosaminuria
  • Fucosidosis
  • Mannosidosis
  • Sialidosis (mucolipidosis I)

Another type, recently characterized, is galactosialidosis.^[3]

External links

• NIH

Notes and References

1. Book: Charles H. Rodeck. Martin J. Whittle. Fetal medicine: basic science and clinical practice. 3 November 2010. 27 October 2008. Elsevier Health Sciences. 978-0-443-10408-4. 362–.
2. Book: Robert V. Stick. Spencer J. Williams. Carbohydrates: the essential molecules of life. 3 November 2010. 2 December 2008. Elsevier. 978-0-240-52118-3. 402–.
3. Bonten EJ, Wang D, Toy JN, etal . Targeting macrophages with baculovirus-produced lysosomal enzymes: implications for enzyme replacement therapy of the glycoprotein storage disorder galactosialidosis . FASEB J. . 18 . 9 . 971–3 . June 2004 . 15084520 . 10.1096/fj.03-0941fje . free .