Human Aminoacylase/ACY1 antibody

Aminoacylase-1, also designated N-acyl-L-amino-acid amidohydrolase or ACY1, is a member of the largest metallopeptidase family called M20A. Aminoacylase-1 is a zinc-binding homodimeric enzyme expressed in kidney, brain, placenta and spleen. It is the most abundant of the aminoacylases. 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. All affected individuals exhibit markedly increased urinary excretion of several N-acetylated amino acids.

Product Specifications

Product Name Alternative

Aminoacylase 1, ACY1D, ACYLASE, Aminoacylase 1

Host

Mouse

Antigen Species

Human

Reactivity

Human

Immunogen

Recombinant human ACY1 (1-408aa) purified from E. coli

Clonality

Monoclonal

Isotype

IgG2b κ

Clone

AT1E2

Conjugation

Unconjugated

Applications

ELISA, WB, ICC/IF, FACS

Purification Method

By protein-A affinity chromatography

Concentration

1 mg/mL (determined by BCA assay)

Additionnal Information

Aminoacylase, ACY1, ATGA0329-10 µg, ATGA0329-20 µg, ATGA0329-50 µg, ATGA0329-100 µg, ATGA0329-250 µg, ATGA0329-500 µg, ATGA0329-1 mg, ATGA0329-10, ATGA0329-20, ATGA0329-50, ATGA0329-100, ATGA0329-250, ATGA0329-500, ATGA0329-1

References & Citations

Cook R.M., et al. (1993) J Biol Chem 268 (23) : 17010-17017.; ; Lindner H.A., et al. (2003) J Biol Chem 278 (45) : 44496-44504.

Storage Conditions

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

Formulation

Liquid in. Phosphate-Buffered Saline (pH 7.4) with 0.02% Sodium Azide, 10% glycerol

Applications Notes

The antibody has been tested by ELISA, Western blot, ICC/IF and FACS analysis to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results.

Scientific Category

Enzymes & Proteases

NCBI Accession Number

NP_000657

Uniprot Accession Number

Q03154

WB Description

The cell lysates of Jurkat, HepG2, Hela, A549 (40ug) were resolved by SDS-PAGE, transferred to PVDF membrane and probed with anti-human ACY1 antibody (1:500) . Proteins were visualized using a goat anti-mouse secondary antibody conjugated to HRP and an ECL detection system. The Recombinant ACY1 protein (50ng) was resolved by SDS-PAGE, transferred to PVDF membrane and probed with anti-human ACY1 antibody (1:1000) . Proteins were visualized using a goat anti-mouse secondary antibody conjugated to HRP and an ECL detection system. {ATGA0329-WB2.jpg}

FACS Description

Flow cytometry analysis of ACY1 in Hep3B cell line, staining at 2-5ug for 1x10^6cells (red line) . The secondary antibody used goat anti-mouse IgG Alexa fluor 488 conjugate. Isotype control antibody was mouse IgG (black line) .

IF Description

ICC/IF analysis of ACY1 in HeLa cells. The cell was stained with ATGA0329 (1:100) . The secondary antibody (green) was used Alexa Fluor 488. DAPI was stained the cell nucleus (blue) .