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Aminoacylase 1 deficiency

Known as: ACY1D, Deficiency of the aminoacylase-1 enzyme 
 
National Institutes of Health

Related topics

Related topics

7 relations
ACY1, ARG197TRPAcute encephalopathyAutosomal recessive inheritanceMuscle Weakness
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Broader (2)

AmidohydrolasesAmino Acid Metabolism, Inborn Errors

Papers overview

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Review
2020
Review
2020
The specific biochemistry of human axilla odour formation viewed in an evolutionary context
Human body odour is dominated by the scent of specific odourants emanating from specialized glands in the axillary region. These… Expand
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2018
2018
Four years follow up of ACY1 deficient patient and pedigree study
Aminoacylase 1 deficiency (ACY1D) is a rare inborn error of metabolism characterized by increased urinary excretion of N… Expand
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2016
2016
Catalytic properties of aminoacylase of strain Rhodococcus armeniensis AM6.1
Studies of substrate specificity revealed that the D-aminoacylase of Rhodococcus armeniensis AM6.1 strain exhibits absolute… Expand
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2013
2013
Cloning, expression and characterization of D-aminoacylase from Achromobacter xylosoxidans subsp. denitrificans ATCC 15173.
D-Aminoacylase catalyzes the conversion of N-acyl-D-amino acids to d-amino acids and fatty acids. The aim of this study was to… Expand
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2013
2013
Characterization and application of a L-specific amino acid oxidase from Rhodococcus sp. AIU LAB-3.
An L-specific amino acid oxidase (L-AAO) suitable for assay of N-acyl-L-amino acid amidohydrolase (L-aminoacylase) activity was… Expand
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2006
2006
Gas chromatographic-mass spectrometric analysis of N-acetylated amino acids: the first case of aminoacylase I deficiency.
During the metabolic work-up of a patient presenting with neonatal convulsions, we consistently observed the presence of unusual… Expand
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2001
2001
Simultaneous analysis of enantiomeric composition of amino acids and N-acetyl-amino acids by enantioselective chromatography.
Among the three chiral columns, CHIROBIOTIC T, CHIRLPAK WH, and CHIRALCEL OD-R, tested for the separation of racemic amino acids… Expand
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1996
1996
Overproduction of D-aminoacylase from Alcaligenes xylosoxydans subsp. xylosoxydans A-6 in Escherichia coli and its purification.
We constructed the high-expression plasmid for D-aminoacylase from Alcaligenes xylosoxydans subsp. xylosoxydans A-6. The… Expand
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1992
1992
ENANTIOSELECTIVE DEPROTECTION OF N-PROTECTED AMINO ACIDS BY D-AMINOACYLASE
Abstract D-Aminoacylase isolated from Alcaligenes faecalis DA1 could enantioselectively deprotect racemic N-protected [such as… Expand
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1992
1992
Production and immobilization of D-aminoacylase of Alcaligenes faecalis DA1 for optical resolution of N-acyl-DL-amino acids.
The production of D-aminoacylase by Alcaligenes faecalis DA1 was induced 5- to 50-fold by N-acetyl-D-amino acids. This strain… Expand
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