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| Product Name | Aminoacylase 1, aa1-408, Recombinant, Human (ACY1) |
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| Description | Purity ~90% (SDS-PAGE). 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 acylatedaas. 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-acetylatedaas. Source: Recombinant corresponding to aa1-408 of human recombinant ACY1 protein, fused to His-tag at N-terminus, expressed in E.coli. AA Sequence: MGSSHHHHHH SSGLVPRGSH MTSKGPEEEH PSVTLFRQYL RIRTVQPKPD YGAAVAFFEE TARQLGLGCQ KVEVAPGYVV TVLTWPGTNP TLSSILLNSH TDVVPVFKEH WSHDPFEAFK DSEGYIYARG AQDMKCVSIQ YLEAVRRLKV E |
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| Size | 50ug |
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| Concentration | n/a |
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| Applications | n/a |
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| Other Names | n/a |
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| Gene, Accession, CAS # | Accession: NP_000657 |
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| Catalog # | A1374-05D |
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| Order / More Info | Aminoacylase 1, aa1-408, Recombinant, Human (ACY1) from UNITED STATES BIOLOGICAL |
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| Product Specific References | n/a |
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