Aminoacylase/ACY1 (clone AT1E2), anti-human
€0.00
In stock
SKU
ATGA0329
Catalog NumberATGA0329
Isotype
Mouse IgG2b kappa
Applications:
ELISA,WB,ICC/IF,FACS
Datasheet
Questions? Contact us!
Isotype
Mouse IgG2b kappa
Applications:
ELISA,WB,ICC/IF,FACS
Datasheet
Questions? Contact us!
Description
Aminoacylase-1, also designated N-acyl-L-amino-acid amidohydrolase or ACY1, is a member of the largest metallopeptidase family called M20A. Aminoacylase-1 is a zinc-binding homodimeric enzyme expressed in kidney, brain, placenta and spleen. It is the most abundant of the aminoacylases. Defects in ACY1 are the cause of aminoacylase-1 deficiency (ACY1D). ACY1D results in a metabolic disorder manifesting with encephalopathy, unspecific psychomotor delay, psychomotor delay with atrophy of the vermis and syringomyelia, marked muscular hypotonia or normal clinical features. All affected individuals exhibit markedly increased urinary excretion of several N-acetylated amino acids.Alternative Names
Aminoacylase 1, ACY1D, ACYLASE, Aminoacylase 1Concentration
1mg/ml (determined by BCA assay)Concentration
Liquid in. Phosphate-Buffered Saline (pH 7.4) with 0.02% Sodium Azide, 10% glycerolStorage: Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
Aminoacylase-1, also designated N-acyl-L-amino-acid amidohydrolase or ACY1, is a member of the largest metallopeptidase family called M20A. Aminoacylase-1 is a zinc-binding homodimeric enzyme expressed in kidney, brain, placenta and spleen. It is the most abundant of the aminoacylases. Defects in ACY1 are the cause of aminoacylase-1 deficiency (ACY1D). ACY1D results in a metabolic disorder manifesting with encephalopathy, unspecific psychomotor delay, psychomotor delay with atrophy of the vermis and syringomyelia, marked muscular hypotonia or normal clinical features. All affected individuals exhibit markedly increased urinary excretion of several N-acetylated amino acids.Alternative Names
Aminoacylase 1, ACY1D, ACYLASE, Aminoacylase 1Concentration
1mg/ml (determined by BCA assay)Concentration
Liquid in. Phosphate-Buffered Saline (pH 7.4) with 0.02% Sodium Azide, 10% glycerolStorage: Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
| Is Featured? | No |
|---|
Write Your Own Review