2.3.1.78: heparan-alpha-glucosaminide N-acetyltransferase
This is an abbreviated version!
For detailed information about heparan-alpha-glucosaminide N-acetyltransferase, go to the full flat file.
Word Map on EC 2.3.1.78
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2.3.1.78
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lysosomal
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mucopolysaccharidosis
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sanfilippo
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glycosaminoglycans
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naglu
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alpha-n-acetylglucosaminidase
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medicine
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sulfamidase
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n-acetylglucosamine-6-sulfatase
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splice-site
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facies
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nonsyndromic
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pigmentosa
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analysis
- 2.3.1.78
- lysosomal
- mucopolysaccharidosis
-
sanfilippo
- glycosaminoglycans
- naglu
- alpha-n-acetylglucosaminidase
- medicine
-
sulfamidase
- n-acetylglucosamine-6-sulfatase
-
splice-site
-
facies
-
nonsyndromic
- pigmentosa
- analysis
Reaction
Synonyms
acetyl-CoA:alpha-glucosaminide N-acetyltransferase, acetyl-coenzyme A-alpha-glucosaminide N-acetyltransferase, acetyl-coenzyme A:alpha-glucosaminide N-acetyltransferase, acetyl-coenzyme:alpha-D-2-amino-glucosamine transferase, acetyltransferase, alpha-glucosaminide, acetyl–coenzyme A:alpha-glucosaminide N-acetyltransferase, GNAT, heparan sulfate acetyl-CoA: alpha-glucosaminide N-acetyltransferase, heparan sulfate acetyl-CoA:alpha-glucosaminide N-acetyltransferase, HGSNAT, TMEM76, transmembrane protein 76
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2.3.1.78 heparan-alpha-glucosaminide N-acetyltransferaseAdvanced search results
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Localization on EC 2.3.1.78 - heparan-alpha-glucosaminide N-acetyltransferase
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LOCALIZATION 
ORGANISM
UNIPROT
COMMENTARY 
GeneOntology No.
LITERATURE
SOURCE
HGSNAT coimmunoprecipitates with antibodies to ALIX, which is associated with the endosomal sorting complexes required for transport proteins
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membrane-bound, greater part of activity is recovered in microsomes
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additional information
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enzyme is associated to detergent-resistent microdomains of lysosomal membrane
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enzyme is acetylated at the cytoplasmic side of the lysosome and the acetyl group is then transferred to the inside where it is used to acetylate heparan sulfate
the enzyme is synthesized as a catalytically inactive 77-kDa precursor that is transported to the lysosomes via an adaptor protein-mediated pathway that involves conserved tyrosine- and dileucine-based lysosomal targeting signals in its C-terminal cytoplasmic domain with a contribution from a dileucine-based signal in the N-terminal cytoplasmic loop. In the lysosome, the precursor is cleaved into a 29-kDa N-terminal alpha-chain and a 48-kDa C-terminal beta-chain, and assembled into active 440-kDa oligomers. The subunits are held together by disulfide bonds between at least two cysteine residues, Cys123 and Cys434, in the lysosomal luminal loops of the enzyme.
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enzyme is acetylated at the cytoplasmic side of the lysosome and the acetyl group is then transferred to the inside where it is used to acetylate heparan sulfate
