MAAI Antibody (B-2): sc-376042

MAAI Antibody (B-2) is a mouse monoclonal IgG1 antibody that detects maleylacetoacetate isomerase (MAAI) in human samples through various applications including western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry with paraffin-embedded sections (IHCP), and enzyme-linked immunosorbent assay (ELISA). MAAI plays a crucial role in the phenylalanine/phenylacetate degradation pathway by catalyzing the conversion of maleylacetoacetate to fumarylacetoacetate, which is essential for proper metabolism of amino acids and prevention of metabolic disorders such as type I tyrosinemia and phenylketonuria. MAAI is primarily located in the liver, where expression is most abundant, and is also found in lower levels in skeletal muscle and brain tissues, highlighting MAAI′s importance in hepatic function and systemic metabolism. MAAI is a dimeric enzyme that exhibits limited activity with known glutathione S-transferase substrates, indicating a specialized role in detoxification processes within the liver. The gene encoding MAAI is located on human chromosome 14q24.3, and evolutionary conservation is evidenced by sequence similarity to GST-like proteins found in other organisms, such as C. elegans and carnation. Anti-MAAI antibody (B-2) serves as an invaluable tool for researchers studying metabolic pathways and related disorders, providing insights into the functional dynamics of this important enzyme.

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MAAI Antibody (B-2) References:

1. Anti-apolipoprotein A-I autoantibody: characterization of monoclonal autoantibodies from patients with systemic lupus erythematosus.  |  Abe, H., et al. 2001. J Rheumatol. 28: 990-5. PMID: 11361227
2. Maleylacetoacetate isomerase (MAAI/GSTZ)-deficient mice reveal a glutathione-dependent nonenzymatic bypass in tyrosine catabolism.  |  Fernández-Cañón, JM., et al. 2002. Mol Cell Biol. 22: 4943-51. PMID: 12052898
3. The dichloroacetate dilemma: environmental hazard versus therapeutic goldmine--both or neither?  |  Stacpoole, PW. 2011. Environ Health Perspect. 119: 155-8. PMID: 20920954
4. Human polymorphisms in the glutathione transferase zeta 1/maleylacetoacetate isomerase gene influence the toxicokinetics of dichloroacetate.  |  Shroads, AL., et al. 2012. J Clin Pharmacol. 52: 837-49. PMID: 21642471
5. Haplotype variations in glutathione transferase zeta 1 influence the kinetics and dynamics of chronic dichloroacetate in children.  |  Shroads, AL., et al. 2015. J Clin Pharmacol. 55: 50-5. PMID: 25079374
6. Hypersuccinylacetonaemia and normal liver function in maleylacetoacetate isomerase deficiency.  |  Yang, H., et al. 2017. J Med Genet. 54: 241-247. PMID: 27876694
7. Cortical thickness is related to cognitive-motor automaticity and attention allocation in individuals with Alzheimer's disease: a regions of interest study.  |  Longhurst, JK., et al. 2023. Exp Brain Res. 241: 1489-1499. PMID: 37085647
8. Maleic acid is a biomarker for maleylacetoacetate isomerase deficiency; implications for newborn screening of tyrosinemia type 1.  |  van Vliet, K., et al. 2023. J Inherit Metab Dis. 46: 1104-1113. PMID: 37545091
9. Clinical physiology and pharmacology of GSTZ1/MAAI.  |  Stacpoole, PW. 2023. Biochem Pharmacol. 217: 115818. PMID: 37742772
10. Characterization of a fungal maleylacetoacetate isomerase gene and identification of its human homologue.  |  Fernández-Cañón, JM. and Peñalva, MA. 1998. J Biol Chem. 273: 329-37. PMID: 9417084

The preferred MAAI antibody is MAAI Antibody (H-1): sc-271411.