Congenital disorders of glycosylation are a growing class of rare diseases. Which organelle would be most commonly involved in the glycoprotein disorder portion of the group?

Golgi apparatus.

Explanation:

Glycosylation is the reaction in which a carbohydrate, i.e. a glycosyl donor, is attached to a hydroxyl or other functional group of another molecule. In biology, glycosylation mainly refers in particular to the enzymatic process that attaches glycans to proteins, or other organic molecules.

The Golgi apparatus is the cell organelle responsible for the modification of the protein before sorting and secretion. They play a major role by modifying the protein by adding short sugar chains. Hence Golgi apparatus would be involved in any disorders of glycosylation.

Golgi apparatus.

Explanation:

Congenital disorders of glycosylation is a disorder caused  by  failure to synthesise the carbohydrate  chain (glycans) or the lipids structure of glycoprotein or glycolipds  respectively  on one part, and  failure to attach these structures to protein and lipids on the other part.

It is caused by mutation in the proteins that  coded for  enzymes needed in catalytic process; of glycosylation, it can be genetic, and different types exists.

Generally  synthesised protein in the R.ER to be secreted out of the cell or be part of the membrane proteins must  be glycosylated, in the Golgi apparatus. Glycosylation   of  involves the translocation of  N-terminal end of the protein, and the  bonding of pre-assembled oligosacchrides to it.(thus in glycoprotein this should be (Glycans +Protein).

Therefore failure of the N-terminal portion of protein to be synthesised, or non -attachment to the oligosachrides structure  in the Golgi Apparatus leads to this congenital disorder. The Golgi  apparatus is the factory for sorting , modification and packaging of protein synthesize by the R.ER, thus it the site of Glycosylation therefore the most relevant  organelle.

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