Description
system_update_alt Datasheet
Product Name: | ACY1 Antibody |
Product Sku: | CPAB0194 |
Size: | 5μg |
Host Species: | Mouse |
Immunogen: | Anti-human ACY1 mAb, is derived from hybridization of mouse F myeloma cells with spleen cells from BALB/c mice immunized with recombinant human ACY1 amino acids 1-48 purified from Ecoli. |
Clone: | PAT1E2AT. |
Reactivity: | Human |
Applications: | Western Blot, ELISA |
Purification Method: | ACY1 antibody was purified from mouse ascitic fluids by protein-A affinity chromatography. |
Isotype: | IgG2b |
Background: | 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 take part in the catabolism and salvage of acylated amino acids. Defects in ACY1 are the source 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. |
Synonyms: | ACY1D, ACYLASE, ACY-1, EC 3.5.1.143. |
Storage Buffer: | For periods up to 1 month store at 4°C, for longer periods of time, store at -20°C. Prevent freeze thaw cycles. |