ACY1 (Aminoacylase 1, Aminoacylase-1, ACY-1, N-acyl-L-amino-acid Amidohydrolase)

Size

£685.00

SKU:: 122956
Purity:: Purified by Protein A affinity chromatography.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Mouse
Immunogen:: Full length recombinant corresponding to aa1-408 from human ACY1 (AAH00545) with GST tag. MW of the GST tag alone is 26kD.
Clone:: 4F1-B7
Format:: Supplied as a liquid in PBS, pH 7.2.
Extra Details:: 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. Applications: Suitable for use in Immunofluorescence, ELISA, Western Blot, Immunoprecipitation and Immunohistochemistry. Other applications not tested. Recommended Dilution: Immunofluorescence: 10ug/ml Immunohistochemistry (Formalin fixed paraffin embedded): 3ug/ml Optimal dilutions to be determined by the researcher. AA Sequence: MTSKGPAEEHPSVTLFRQYLRIRTVQPKPDYGAAVAFFEETARQLGLGCQKVEVAPGYVVTVLTWPGTNPTLSSILLNSHTDVVPVFKEHWSHDPFEAFKDSEGYIYARGAQDVKCVSIQYLEAVRRLKVEGHRFPRTIHMTFVPDEEVGGHQGMELFVQRPEFHALRAGFALDEGIANPTDAFTVFYSERSPWWVRVTSTGRPGHASRFMEDTAAEKLHKVVNSILAFREKEWQRLQSNPHLKEGSVTSVNLTKLEGGVAYNVIPATMSASFDFRVAPDVDFKAFEEQLQSWCQAAGEGVTLEFAQKWMHPQVTPTDDSNPWWAAFSRVCKDMNLTLEPEIMPAATDNRYIRAVGVPALGFSPMNRTPVLLHDHDERLHEAVFLRGVDIYTRLLPALASVPALPSDS Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Shipping Conditions:: Blue Ice