α-N-acetylglucosaminidase (, ''α-acetylglucosaminidase'', ''N-acetyl-α-D-glucosaminidase'', ''N-acetyl-α-glucosaminidase'', ''α-D-2-acetamido-2-deoxyglucosidase'') is a
protein
Proteins are large biomolecules and macromolecules that comprise one or more long chains of amino acid residues. Proteins perform a vast array of functions within organisms, including catalysing metabolic reactions, DNA replication, res ...
associated with
Sanfilippo syndrome
Sanfilippo syndrome, also known as mucopolysaccharidosis type III (MPS III), is a rare autosomal recessive lysosomal storage disease that primarily affects the brain and spinal cord. It is caused by a buildup of large sugar molecules called g ...
. It is an
enzyme
Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as products ...
with
systematic name A systematic name is a name given in a systematic way to one unique group, organism, object or chemical substance, out of a specific population or collection. Systematic names are usually part of a nomenclature.
A semisystematic name or semitrivial ...
''α-N-acetyl-D-glucosaminide N-acetylglucosaminohydrolase''.
This enzyme
catalyses the following
chemical reaction
A chemical reaction is a process that leads to the IUPAC nomenclature for organic transformations, chemical transformation of one set of chemical substances to another. Classically, chemical reactions encompass changes that only involve the pos ...
:
Hydrolysis
Hydrolysis (; ) is any chemical reaction in which a molecule of water breaks one or more chemical bonds. The term is used broadly for substitution, elimination, and solvation reactions in which water is the nucleophile.
Biological hydrolys ...
of terminal non-reducing N-acetyl-D-glucosamine residues in N-acetyl-α-D-glucosaminides
This enzyme hydrolyses
UDP-N-acetylglucosamine
Uridine diphosphate ''N''-acetylglucosamine or UDP-GlcNAc is a nucleotide sugar and a coenzyme in metabolism. It is used by glycosyltransferases to transfer ''N''-acetylglucosamine residues to substrates. D-Glucosamine is made naturally in the f ...
.
References
External links
*
EC 3.2.1
{{3.2-enzyme-stub