Mouse ACY1 Monoclonal Antibody | anti-ACY1 antibody

ACY1 antibody

Cat. Num. AAA533326

• Gene Names: ACY1; ACY-1; ACY1D; HEL-S-5
• Applications: Flow Cytometry, Functional Assay, Immunohistochemistry, Western Blot
• Purity: ACY1 antibody was purified by affinity chromatography.
• Synonyms: ACY1; Monoclonal Antibody; ACY1 antibody; Monoclonal ACY1; Anti-ACY1; ACY1D; ACYLASE; Aminoacylase 1; ACY 1; ACY-1; anti-ACY1 antibody

• Host: Mouse
• Clonality: Monoclonal
• Isotype: IgG1
• Clone Number: 1B12
• Purity/Purification: ACY1 antibody was purified by affinity chromatography.
• Form/Format: Supplied in PBS buffer, pH 7.3, containing 1% BSA, 50% glycerol and 0.02% sodium azide.
• Concentration: 500 ug-1 mg/ml (varies by lot)
• Sequence Length: 408

• Applicable Applications for anti-ACY1 antibody: Flow Cytometry (FC/FACS), Immunohistochemistry (IHC), Western Blot (WB)
• Application Notes: FC: 1:100; IHC: 1:150; WB: 1:200-500
• Biological Significance: ACY1 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 urin ary excretion of several N-acetylated amino acids.
• Cross-Reactivity: Human, Dog, Rat, Mouse
• Immunogen: ACY1 antibody was raised in mouse using a full length recombinant protein of human ACY1 (NP_000657) produced in HEk293 cell as the immunogen.
• Preparation and Storage: Store at 4 degree C for short term storage. Aliquot and store at -20 degree C for long term storage. Avoid repeated freeze/thaw cycles.

Western Blot (WB)

(Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant ACY1 protein (Right) or empty vector (Left) detected with ACY1 antibody)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of ACY1 protein in paraffin embedded Human liver tissue using ACY1 antibody)

Related Product Information for anti-ACY1 antibody

Mouse monoclonal ACY1 antibody

Product Categories/Family for anti-ACY1 antibody

Proteases; Inhibitors; & Enzymes

NCBI and Uniprot Product Information

• NCBI GI #: 4501901
• NCBI GeneID: 95
• NCBI Accession #: NP_000657
• NCBI GenBank Nucleotide #: NM_000666.2
• UniProt Accession #: Q03154; C9J6I6; C9J9D8; C9JWD4
• Molecular Weight: 42,195 Da
• NCBI Official Full Name: aminoacylase-1 isoform a
• NCBI Official Synonym Full Names: aminoacylase 1
• NCBI Official Symbol: ACY1
• NCBI Official Synonym Symbols: ACY-1; ACY1D; HEL-S-5
• NCBI Protein Information: aminoacylase-1
• UniProt Protein Name: Aminoacylase-1
• Protein Family: Aminoacylase
• UniProt Gene Name: ACY1
• UniProt Synonym Gene Names: ACY-1
• UniProt Entry Name: ACY1_HUMAN

NCBI Description

This gene encodes a cytosolic, homodimeric, zinc-binding enzyme that catalyzes the hydrolysis of acylated L-amino acids to L-amino acids and an acyl group, and has been postulated to function in the catabolism and salvage of acylated amino acids. This gene is located on chromosome 3p21.1, a region reduced to homozygosity in small-cell lung cancer (SCLC), and its expression has been reported to be reduced or undetectable in SCLC cell lines and tumors. The amino acid sequence of human aminoacylase-1 is highly homologous to the porcine counterpart, and this enzyme is the first member of a new family of zinc-binding enzymes. Mutations in this gene cause aminoacylase-1 deficiency, a metabolic disorder characterized by central nervous system defects and increased urinary excretion of N-acetylated amino acids. Alternative splicing of this gene results in multiple transcript variants. Read-through transcription also exists between this gene and the upstream ABHD14A (abhydrolase domain containing 14A) gene, as represented in GeneID:100526760. A related pseudogene has been identified on chromosome 18. [provided by RefSeq, Nov 2010]

Uniprot Description

ACY1: Involved in the hydrolysis of N-acylated or N-acetylated amino acids (except L-aspartate). 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. Belongs to the peptidase M20A family.

Protein type: Hydrolase; EC 3.5.1.14; Amino Acid Metabolism - arginine and proline

Chromosomal Location of Human Ortholog: 3p21.1

Cellular Component: cytosol

Molecular Function: metallopeptidase activity; metal ion binding; aminoacylase activity

Biological Process: amino acid metabolic process; xenobiotic metabolic process; proteolysis

Disease: Aminoacylase 1 Deficiency