protein

Recombinant human Aminoacylase/ACY1 protein

ATGP0659

10 ug

105 USD

Recombinant human Aminoacylase/ACY1 protein

1-408aa

ATGP0659

NP_000657.1

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.

Aminoacylase 1, ACY1D, ACYLASE, Aminoacylase 1

0.5mg/ml (determined by Bradford assay)

Liquid in. Phosphate-Buffered Saline (pH 7.4) containing 10% glycerol

Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Sass J.O., et al. (2006) Am. J. Hum. Genet. 78:401-409;; Van Coster RN., et al. (2005) Biochem Biophys Res Commun. 338(3):1322-6;;

> 90% by SDS-PAGE

SDS-PAGE

Human

Enzymes & Proteases

48kDa (428aa) confirmed by MALDI-TOF

TSKGPEEEH PSVTLFRQYL RIRTVQPKPD YGAAVAFFEE TARQLGLGCQ KVEVAPGYVV TVLTWPGTNP TLSSILLNSH TDVVPVFKEH WSHDPFEAFK DSEGYIYARG AQDMKCVSIQ YLEAVRRLKV EGHRFPRTIH MTFVPDEEVG GHQGMELFVQ RPEFHALRAG FALDEGIANP TDAFTVFYSE RSPWWVRVTS TGRPGHASRF MEDTAAEKLH KVVNSILAFR EKEWQRLQSN PHLKEGSVTS VNLTKLEGGV AYNVIPATMS ASFDFRVAPD VDFKAFEEQL QSWCQAAGEG VTLEFAQKWM HPQVTPTDDS NPWWAAFSRV CKDMNLTLEP EIMPAATDNR YIRAVGVPAL GFSPMNRTPV LLHDHDERLH EAVFLRGVDI YTRLLPALAS VPALPSDS

His-Tag

E.coli

Recombinant Protein

Q03154

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