Human biochem lecture 7

Glycogen

A storage molecule that strings and folds glucose into readily available but tightly bound units.

Glycogen Storage Diseases (GSDs)

Metabolic diseases caused by deficiencies in the enzymes involved in glycogen storage and release.

Von Gierke's disease (GSD1a)

The most common glycogen storage disease, caused by a deficiency in glucose-6-phosphatase enzyme.

Monosaccharides

The simplest carbohydrate molecules, composed of a single structural unit.

Aldoses

Monosaccharides that have a double bonded oxygen on the end of the carbon chain.

Ketoses

Monosaccharides that have a double bonded oxygen in the middle of the carbon chain.

Enantiomers

Compounds that have the same composition but are mirror images of each other.

D-enantiomers

The configuration of monosaccharides that are commonly utilized in metabolism.

Diastereomers

Compounds that have the same number of atoms but differ in the arrangement of those atoms.

Polysaccharides

Carbohydrates that consist of long chains of monosaccharides, used for storage or structure.

Amylose

A storage polysaccharide made of unbranched chains of glucose linked by alpha-1,4 linkages.

Amylopectin

A storage polysaccharide with branched chains of glucose linked by both alpha-1,4 and alpha-1,6 linkages.

Cellulose

A structural polysaccharide formed by beta-1,4 linkages of glucose, contributing to plant cell wall strength.

Glycoproteins

Proteins that have oligosaccharides or polysaccharides attached, serving various biological functions.

N-linked glycoproteins

Glycoproteins where oligosaccharides are attached to the amide group of asparagine residues.

O-linked glycoproteins

Glycoproteins where oligosaccharides are attached to hydroxyl groups of serine or threonine residues.