Search results for ACY1

Showing 13 results out of 13

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Species

Types

Compartments

Reaction types

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Protein (6 results from a total of 6)

Identifier: R-HSA-5433075
Species: Homo sapiens
Compartment: cytosol
Primary external reference: UniProt: ACY1: Q03154
Identifier: R-HSA-5579111
Species: Homo sapiens
Compartment: cytosol
Primary external reference: UniProt: ACY1: Q03154
Identifier: R-HSA-5579041
Species: Homo sapiens
Compartment: cytosol
Primary external reference: UniProt: ACY1: Q03154
Identifier: R-HSA-5579040
Species: Homo sapiens
Compartment: cytosol
Primary external reference: UniProt: ACY1: Q03154
Identifier: R-HSA-5579106
Species: Homo sapiens
Compartment: cytosol
Primary external reference: UniProt: ACY1: Q03154
Identifier: R-HSA-5579105
Species: Homo sapiens
Compartment: cytosol
Primary external reference: UniProt: ACY1: Q03154

Set (1 results from a total of 1)

Identifier: R-HSA-5579044
Species: Homo sapiens
Compartment: cytosol

Complex (2 results from a total of 2)

Identifier: R-HSA-9638048
Species: Homo sapiens
Compartment: cytosol
Identifier: R-HSA-5433068
Species: Homo sapiens
Compartment: cytosol

Pathway (1 results from a total of 1)

Identifier: R-HSA-5579007
Species: Homo sapiens
Aminoacylase 1 (ACY1) is a cytosolic, homodimeric zinc-binding metalloenzyme with a wide range of tissue expression. It hydrolyses acylated L-amino acids (except L-aspartate) into L-amino acids and an acyl group. It can also hydrolyse N-acetylcysteine-S-conjugates. Defects in ACY1 can cause aminoacylase-1 deficiency (ACY1D; MIM:609924) resulting in encephalopathy, delay in psychomotor development, seizures and increased urinary excretion of several N-acetylated amino acids (Sass et al. 2006, Sass et al. 2007).

Reaction (3 results from a total of 3)

Identifier: R-HSA-5579081
Species: Homo sapiens
Compartment: cytosol
Cytosolic aminocyclases 1 and 3 (ACY1,3) can hydrolyse N-acylated amino acids and N-acylcysteine-S-conjugates (Lindner et al. 2003). They are functional as dimers and utilise zinc as a cofactor. Defects in ACY1 can cause aminoacylase 1 deficiency (ACY1D; MIM:609924), leading to acute encephalopathy, seizures, impaired psychomotor development and increased urinary excretion of several N-acetylated amino acids. The missense mutation R353C is associated with recessive loss-of-function phenotype and is the most common cause of enzyme deficiency (Sass et al. 2006, Sass et al. 2007).
Identifier: R-HSA-9753944
Species: Homo sapiens
Compartment: cytosol
The effects of paracetamol (APAP) poisoning can be reversed by administration of N-acetyl-L-cysteine (NAC) within 15hrs of APAP intake. This treatment is effective in preventing liver damage, hepatic failure, renal damage, and death (Prescott et al. 1977, Prescott 1981). NAC provides L-cysteine (L-Cys) for the formation of the tripeptide glutathione, which can conjugate the reactive APAP metabolite N-acetyl-p-benzoquinine imine (NAPQI), thereby reducing damage. NAC is thought to be deacetylated to L-Cys by aminocylase 1 (ACY1), a dimeric enzyme highly expressed in the kidneys (Stocker et al. 2012, review Pedre et al. 2021).
Identifier: R-HSA-5433074
Species: Homo sapiens
Compartment: cytosol
Cytosolic aminocyclases 1 and 3 (ACY1,3) can hydrolyse N-acylated amino acids and N-acylcysteine-S-conjugates (Lindner et al. 2003). They are functional as dimers and utilise zinc as a cofactor.
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