ACY1 Polyclonal Antibody

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.,