Chitobiosyldiphosphodolichol beta-mannosyltransferase
Chitobiosyldiphosphodolichol beta-mannosyltransferase is an enzyme that plays a crucial role in the process of N-linked glycosylation in eukaryotic cells. This enzyme is responsible for the transfer of a mannose residue from GDP-mannose to chitobiosyldiphosphodolichol, forming a key intermediate in the synthesis of the lipid-linked oligosaccharide precursor.
Function[edit | edit source]
Chitobiosyldiphosphodolichol beta-mannosyltransferase is involved in the early steps of the N-linked glycosylation pathway. This pathway is essential for the proper folding and function of many glycoproteins. The enzyme catalyzes the addition of a mannose residue to the growing oligosaccharide chain attached to the lipid carrier dolichol. This step is critical for the subsequent elongation and processing of the oligosaccharide precursor.
Mechanism[edit | edit source]
The enzyme uses GDP-mannose as a donor substrate and transfers the mannose residue to the chitobiosyldiphosphodolichol acceptor. The reaction occurs in the endoplasmic reticulum membrane, where the lipid-linked oligosaccharide is assembled before being transferred to nascent polypeptides.
Biological Importance[edit | edit source]
The activity of chitobiosyldiphosphodolichol beta-mannosyltransferase is essential for the proper assembly of the N-linked glycan precursor. Defects in this enzyme can lead to disorders in glycosylation, which can result in a variety of congenital disorders of glycosylation (CDGs). These disorders often have severe clinical manifestations, including developmental delays, neurological issues, and other systemic problems.
Related Enzymes[edit | edit source]
Chitobiosyldiphosphodolichol beta-mannosyltransferase is part of a larger family of glycosyltransferases that are involved in the synthesis of complex carbohydrates. Other related enzymes include:
See Also[edit | edit source]
- N-linked glycosylation
- Glycosyltransferase
- Dolichol
- Endoplasmic reticulum
- Congenital disorders of glycosylation
References[edit | edit source]
External Links[edit | edit source]
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Contributors: Prab R. Tumpati, MD
