产品详情 |
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Antigenic Specificity | ACY1 |
Clone | monoclonal |
Host Species | Mouse |
Reactive Species | human |
Isotype | IgG2b,k |
Format | purified |
Size | 100ul, 50ul |
Concentration | n/a |
Applications | ELISA, WB, Flow cytometry, ICC/IF |
Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
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. |
Immunogen | Recombinant human ACY1 (1-408aa) purified from E. coli |
Other Names | Aminoacylase 1, ACY1D, ACYLASE, Aminoacylase 1 |
Gene, Accession # | NP 000657 |
Catalog # | ATGA0329 |
Price | |
Order / More Info | ACY1 Antibody from ATGen |
Product Specific References | n/a |
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