Glycoproteinosis

Glycoproteinosis

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Glycoproteinosis (or "glycoprotein storage disorders") are lysosomal storage diseases affecting glycoproteins, resulting from defects in lysosomal function.

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.cite journal |author=Bonten EJ, Wang D, Toy JN, "et al" |title=Targeting macrophages with baculovirus-produced lysosomal enzymes: implications for enzyme replacement therapy of the glycoprotein storage disorder galactosialidosis |journal=FASEB J. |volume=18 |issue=9 |pages=971–3 |year=2004 |month=June |pmid=15084520 |doi=10.1096/fj.03-0941fje |url=http://www.fasebj.org/cgi/pmidlookup?view=long&pmid=15084520]

References

External links

* [http://www.ninds.nih.gov/news_and_events/proceedings/GlycoproteinosesWorkshopSummary.htm NIH]
* [http://www.mannosidosis.org/whatIsKnown.htm ISMRD]


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