Anti-Aconitase 1/ACO1 Antibody Picoband® Fluoro488 Conjugated

The protein encoded by this gene is a bifunctional, cytosolic protein that functions as an essential enzyme in the TCA cycle and interacts with mRNA to control the levels of iron inside cells. When cellular iron levels are high, this protein binds to a 4Fe-4S cluster and functions as an aconitase. Aconitases are iron-sulfur proteins that function to catalyze the conversion of citrate to isocitrate. When cellular iron levels are low, the protein binds to iron-responsive elements (IREs), which are stem-loop structures found in the 5' UTR of ferritin mRNA, and in the 3' UTR of transferrin receptor mRNA. When the protein binds to IRE, it results in repression of translation of ferritin mRNA, and inhibition of degradation of the otherwise rapidly degraded transferrin receptor mRNA. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. Alternative splicing results in multiple transcript variants.

Part of the TCR-CD3 complex present on T-lymphocyte cell surface that plays an essential role in adaptive immune response. When antigen presenting cells (APCs) activate T-cell receptor (TCR), TCR-mediated signals are transmitted across the cell membrane by the CD3 chains CD3D, CD3E, CD3G and CD3Z. All CD3 chains contain immunoreceptor tyrosine-based activation motifs (ITAMs) in their cytoplasmic domain. Upon TCR engagement, these motifs become phosphorylated by Src family protein tyrosine kinases LCK and FYN, resulting in the activation of downstream signaling pathways. In addition of this role of signal transduction in T-cell activation, CD3E plays an essential role in correct T-cell development (PubMed:19956738, PubMed:24899501) . Participates also in internalization and cell surface down-regulation of TCR-CD3 complexes via endocytosis sequences present in CD3E cytosolic region.

1. Azevedo, E. S., Da Silva, M. C. B. O., Lima, A. M. V., Fonseca, E. F., Conseicao, M. M. Human aconitase polymorphism in three samples from northeastern Brazil. Ann. Hum. Genet. 43: 7-10, 1979. 2. Condo, I., Malisan, F., Guccini, I., Serio, D., Rufini, A., Testi, R. Molecular control of the cytosolic aconitase/IRP1 switch by extramitochondrial frataxin. Hum. Molec. Genet. 19: 1221-1229, 2010. 3. Eisenstein, R. S. Iron regulatory proteins and the molecular control of mammalian iron metabolism. Annu. Rev. Nutr. 20: 627-662, 2000.

Alpha-beta T cell receptor complex; external side of plasma membrane; immunological synapse; cell body; cell-cell junction; dendritic spine; integral component of membrane