Molecular weight (DA)
44kD
Immunogen
Synthesized peptide derived from part region of human protein
Specificity
ACY1 Polyclonal Antibody detects endogenous levels of protein.
Formulation
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
Dilution rate
WB 1:500-2000 ELISA 1:5000-20000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
This gene encodes a cytosolic, homodimeric, zinc-binding enzyme that catalyzes the hydrolysis of acylated L-amino acids to L-amino acids and an acyl group, and has been postulated to function in the catabolism and salvage of acylated amino acids. This gene is located on chromosome 3p21.1, a region reduced to homozygosity in small-cell lung cancer (SCLC), and its expression has been reported to be reduced or undetectable in SCLC cell lines and tumors. The amino acid sequence of human aminoacylase-1 is highly homologous to the porcine counterpart, and this enzyme is the first member of a new family of zinc-binding enzymes. Mutations in this gene cause aminoacylase-1 deficiency, a metabolic disorder characterized by central nervous system defects and increased urinary excretion of N-acetylated amino acids. Alternative splicing of this gene results in multiple transcript variants. Read-through transcription als
Function
catalytic activity:An N-acyl-L-amino acid + H(2)O = a carboxylate + an L-amino acid.,cofactor:Binds 2 zinc ions per subunit.,disease:Defects in ACY1 are the cause of aminoacylase-1 deficiency (ACY1D) [MIM:609924]. 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. Epileptic seizures are a frequent feature. All affected individuals exhibit markedly increased urinary excretion of several N-acetylated amino acids.,function:Involved in the hydrolysis of N-acylated or N-acetylated amino acids (except L-aspartate).,similarity:Belongs to the peptidase M20A family.,subunit:Homodimer. Interacts with SPHK1.,tissue specificity:Expression is highest in kidney, strong in brain and weaker in placenta and spleen.,
Protein name
Aminoacylase-1 (ACY-1) (EC 3.5.1.14) (N-acyl-L-amino-acid amidohydrolase)
Fields
>>Arginine biosynthesis;>>Metabolic pathways;>>2-Oxocarboxylic acid metabolism;>>Biosynthesis of amino acids
Human protein sequence Database
Q03154
Mouse protein sequence database
Q99JW2
Rat protein sequence database
Cellular localization
Cytoplasm.
Tissue expression
Expression is highest in kidney, strong in brain and weaker in placenta and spleen.
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