Anti-AACT/SERPINA3 Antibody Picoband® Fluoro488 Conjugated

Alpha 1-antichymotrypsin, also called serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 3 or GIG24 is an alpha globulin glycoprotein that is a member of the serpin superfamily. In humans, it is encoded by the SERPINA3 gene. This gene is mapped to 14q32.13. The protein encoded by this gene is a plasma protease inhibitor and member of the serine protease inhibitor class. Polymorphisms in this protein appear to be tissue specific and influence protease targeting. Variations in this protein's sequence have been implicated in Alzheimer's disease, and deficiency of this protein has been associated with liver disease. Mutations have been identified in patients with Parkinson disease and chronic obstructive pulmonary disease.

Highly expressed in cardiac, skeletal muscle and during myoblast differentiation. Weakly expressed in other tissues (at protein level) . Expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.

Cancer, Cardiovascular, Cholesterol Metabolism, Endocrine System, G Protein Signaling, Lipid and Lipoprotein Metabolism, Lipid Metabolism, Lipids/Lipoproteins, Metabolic Signaling Pathways, Metabolism, More Gpcr, Neuroscience, Neurotransmission, Pathways and Processes, Receptors / Channels, Signal Transduction, Signaling Pathway

Mediates pre-mRNA alternative splicing regulation. Acts either as activator or repressor of splicing on specific pre-mRNA targets. Inhibits cardiac troponin-T (TNNT2) pre-mRNA exon inclusion but induces insulin receptor (IR) pre-mRNA exon inclusion in muscle. Antagonizes the alternative splicing activity pattern of CELF proteins. Regulates the TNNT2 exon 5 skipping through competition with U2AF2. Inhibits the formation of the spliceosome A complex on intron 4 of TNNT2 pre-mRNA. Binds to the stem-loop structure within the polypyrimidine tract of TNNT2 intron 4 during spliceosome assembly. Binds to the 5'-YGCU (U/G) Y-3'consensus sequence. Binds to the IR RNA. Binds to expanded CUG repeat RNA, which folds into a hairpin structure containing GC base pairs and bulged, unpaired U residues.

1. Chappell, S., Daly, L., Morgan, K., Baranes, T. G., Roca, J., Rabinovich, R., Millar, A., Donnelly, S. C., Keatings, V., MacNee, W., Stolk, J., Hiemstra, P., Miniati, M., Monti, S., O'Connor, C. M., Kalsheker, N. Cryptic haplotypes of SERPINA1 confer susceptibility to chronic obstructive pulmonary disease. Hum. Mutat. 27: 103-109, 2006. 2. Wang, X., DeKosky, S. T., Luedecking-Zimmer, E., Ganguli, M., Kamboh, M. I. Genetic variation in alpha-1-antichymotrypsin and its association with Alzheimer's disease. Hum. Genet. 110: 356-365, 2002.