Glycoproteinosis
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Glycoproteinosis are lysosomal storage diseases affecting
glycoproteins Glycoproteins are proteins which contain oligosaccharide (sugar) chains covalently attached to amino acid side-chains. The carbohydrate is attached to the protein in a cotranslational or posttranslational modification. This process is known a ...
, resulting from defects in lysosomal function. The term is sometimes reserved for conditions involving degradation of glycoproteins. __TOC__


Types

* (E77.0) Defects in
post-translational modification In molecular biology, post-translational modification (PTM) is the covalent process of changing proteins following protein biosynthesis. PTMs may involve enzymes or occur spontaneously. Proteins are created by ribosomes, which translation (biolog ...
of lysosomal enzymes **
Mucolipidosis Mucolipidosis is a group of inherited metabolic disorders that affect the body's ability to carry out the normal turnover of various materials within cells. When originally named, the mucolipidoses derived their name from the similarity in p ...
II (I-cell disease) **
Mucolipidosis Mucolipidosis is a group of inherited metabolic disorders that affect the body's ability to carry out the normal turnover of various materials within cells. When originally named, the mucolipidoses derived their name from the similarity in p ...
III (pseudo-Hurler polydystrophy) * (E77.1) Defects in
glycoprotein Glycoproteins are proteins which contain oligosaccharide (sugar) chains covalently attached to amino acid side-chains. The carbohydrate is attached to the protein in a cotranslational or posttranslational modification. This process is known a ...
degradation ** Aspartylglucosaminuria ** Fucosidosis ** Mannosidosis **
Sialidosis Mucolipidosis type I (ML I) is an inherited lysosomal storage disease that results from a deficiency of the enzyme alpha-N -acetyl neuraminidase (sialidase). The lack of this enzyme results in an abnormal accumulation of complex carbohydrates kno ...
(
mucolipidosis Mucolipidosis is a group of inherited metabolic disorders that affect the body's ability to carry out the normal turnover of various materials within cells. When originally named, the mucolipidoses derived their name from the similarity in p ...
I) Another type, recently characterized, is galactosialidosis.


References


External links


NIH
Glycoprotein metabolism disorders {{endocrine-disease-stub