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
ECTree
2.3.1.78 heparan-alpha-glucosaminide N-acetyltransferaseAdvanced search results
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results (Natural Substrates Products
Natural Substrates Products on EC 2.3.1.78 - heparan-alpha-glucosaminide N-acetyltransferase
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NATURAL SUBSTRATE 
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
acetyl-CoA + heparan sulfate alpha-D-glucosaminide
CoA + heparan sulfate N-acetyl-alpha-D-glucosaminide
acetyl-CoA + heparan sulfate alpha-D-glucosaminide
CoA + heparan sulfate N-acetyl-alpha-D-glucosaminide
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?
acetyl-CoA + heparan sulfate alpha-D-glucosaminide
CoA + heparan sulfate N-acetyl-alpha-D-glucosaminide
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initial step in heparan sulfate degradation
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?
acetyl-CoA + heparan sulfate alpha-D-glucosaminide
CoA + heparan sulfate N-acetyl-alpha-D-glucosaminide
mucopolysaccharidosis IIIC (MPS IIIC, or Sanfilippo C syndrome) is a lysosomal storage disorder caused by the inherited deficiency of the lysosomal membrane enzyme acetyl-coenzyme A:alpha-glucosaminide N-acetyltransferase, which leads to impaired degradation of heparan sulfate. Structural protein that transports the activated acetyl residues across the cell membrane
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?
acetyl-CoA + heparan sulfate alpha-D-glucosaminide
CoA + heparan sulfate N-acetyl-alpha-D-glucosaminide
mucopolysaccharidosis IIIC (MPS IIIC, or Sanfilippo C syndrome) is a lysosomal storage disorder caused by the inherited deficiency of the lysosomal membrane enzyme acetyl-coenzyme A:alpha-glucosaminide N-acetyltransferase, which leads to impaired degradation of heparan sulfate. Structural protein that transports the activated acetyl residues across the cell membrane
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?
acetyl-CoA + heparan sulfate alpha-D-glucosaminide
CoA + heparan sulfate N-acetyl-alpha-D-glucosaminide
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?
additional information
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enzyme deficiency leads to Sanfilippo syndrome type C, i.e. mucopolysaccharidosis III C
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additional information
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enzyme deficiency leads to Sanfilippo syndrome type C, i.e. mucopolysaccharidosis III C
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?
additional information
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enzyme deficiency leads to Sanfilippo syndrome type C, i.e. mucopolysaccharidosis III C
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additional information
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enzyme deficiency causes mucopolysaccharidosis type IIIC
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additional information
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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
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additional information
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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
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?
