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小鼠 Aminoacylase-1/ACY1  抗体

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Aminoacylase-1/ACY1
反应性: Mouse  
应用 : ELISA  
50008-RP01-400
50008-RP01-200
50008-RP01-100
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    反应性: Mouse  
    应用 : 
    50008-T60-50
    50008-T60-200
    50008-T60-100
    50 µg 
    200 µg 
    100 µg 
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    50008-M08HL-300 

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    Aminoacylase-1/ACY1 相关研究领域

    Aminoacylase-1/ACY1 相关信号通路

      Aminoacylase-1/ACY1 相关蛋白、抗体、cDNA基因、ELISA试剂盒

      Aminoacylase-1/ACY1 相关蛋白、抗体、cDNA基因、ELISA试剂盒

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      Aminoacylase-1/ACY1 概述&蛋白信息

      Aminoacylase-1/ACY1 研究背景

      基因概述: 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.
      General information above from NCBI
      催化活性: An N-acyl-aliphatic-L-amino acid + H(2)O = an aliphatic L-amino acid + a carboxylate. {ECO:0000269|PubMed:12933810}.; An N-acetyl-L-cysteine-S-conjugate + H(2)O = an L-cysteine-S-conjugate + acetate. {ECO:0000269|PubMed:12933810}.
      辅因子: Name=Zn(2+); Xref=ChEBI:CHEBI:29105; Evidence={ECO:0000269|PubMed:12933810}; ; Note=Binds 2 Zn(2+) ions per subunit. {ECO:0000269|PubMed:12933810};
      亚单位结构: Homodimer. Interacts with SPHK1 (By similarity). {ECO:0000250}.
      亚细胞定位: Cytoplasm.
      组织特异性: Expression is highest in kidney, strong in brain and weaker in placenta and spleen. {ECO:0000269|PubMed:16465618}.
      相关疾病 : DISEASE: Aminoacylase-1 deficiency (ACY1D) [MIM:609924]: An enzymatic deficiency resulting in 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. {ECO:0000269|PubMed:16274666, ECO:0000269|PubMed:16465618, ECO:0000269|PubMed:17562838, ECO:0000269|PubMed:21414403}. Note=The disease is caused by mutations affecting the gene represented in this entry.
      相似的序列: Belongs to the peptidase M20A family. {ECO:0000305}.
      General information above from UniProt

      Aminoacylase 1 (ACY1), a metalloenzyme that removes amide-linked ACY1 groups from amino acids and may play a role in regulating responses to oxidative stress. Both the C-terminal fragment found in the two-hybrid screen and full-length ACY1 co-immunoprecipitate with SphK1. Though both C-terminal and full-length proteins slightly reduce SphK1 activity measured in vitro, the C-terminal fragment inhibits while full-length ACY1 potentiates the effects of SphK1 on proliferation and apoptosis. It suggested that ACY1 physically interacts with SphK1 and may influence its physiological functions. As a homodimeric zinc-binding enzyme, Aminoacylase 1 catalyzes the hydrolysis of N alpha-acylated amino acids. Deficiency of Aminoacylase 1 due to mutations in the Aminoacylase 1 (ACY1) gene follows an autosomal-recessive trait of inheritance and is characterized by accumulation of N-acetyl amino acids in the urine.

      Aminoacylase-1/ACY1 别称

      ACY-1,ACY1D,HEL-S-5, [homo-sapiens]
      ACY1,ACY-1,ACY1D,ACYLASE,aminoacylase-1, [human]
      1110014J22Rik,Acy1,Acy-1, [mouse]
      Acy-1,1110014J22Rik, [mus-musculus]

      Aminoacylase-1/ACY1 相关文献

    • Sommer A, et al. (2011) The molecular basis of aminoacylase 1 deficiency. Biochim Biophys Acta. 1812(6): 685-90.
    • Maceyka M, et al. (2004) Aminoacylase 1 is a sphingosine kinase 1-interacting protein. FEBS Lett. 568(1-3): 30-4.
    • Cook RM, et al.(1993) Human aminoacylase-1. Cloning, sequence, and expression analysis of a chromosome 3p21 gene inactivated in small cell lung cancer. J Biol Chem. 268(23): 17010-7.
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