122956-BIOTIN
ACY1 (Aminoacylase 1, Aminoacylase-1, ACY-1, N-acyl-L-amino-acid Amidohydrolase) (Biotin)
Size
£927.00
- SKU:
- 122956-BIOTIN
- 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. No preservative added. Labeled with Biotin.
- 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: Store product at 4°C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C. Aliquots are stable at -20°C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Note: Applications are based on unconjugated antibody.
- Shipping Conditions:
- Blue Ice