| 产品详情 |
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| Product Name | ACY1, 1-408aa, Human, His tag, E.coli |
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| Description | Purity: > 90% by SDS - PAGE Format: Liquid. 20mM Tris-HCl buffer (pH8.0) containing 10% glycerol, 1mM DTT . Aminoacylase-1 is a cytosolic, homodimeric, zinc-binding enzyme that catalyzes the hydrolysis of acylated L-amino acids to L-amino acids and acyl group, and has been postulated to function in the catabolism and salvage of acylated amino acids. 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. Epileptic seizures are a frequent feature. All affected individuals exhibit markedly increased urinary excretion of several N-acetylated amino acids. Recombinant human ACY1 protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques. |
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| Size | 250ug, 50ug, 10ug |
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| Concentration | n/a |
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| Applications | SDS-PAGE |
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| Other Names | Aminoacylase 1, ACY1D, ACYLASE, Aminoacylase 1 |
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| Gene, Accession, CAS # | NP 000657 |
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| Catalog # | ATGP0659 |
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| Order / More Info | ACY1, 1-408aa, Human, His tag, E.coli from ATGen |
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| Product Specific References | n/a |
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