Anti-Transcriptional activator GLI3 Gli3 Antibody Picoband®, FITC Conjugate

GLI3 (Gli-kruppel family member 3), also called ONCOGENE GLI3, encodes a member of the zinc finger gene family related to Kruppel, a gene that is known to regulate development in Drosophila. The GLI3 gene is expressed as an 8.5-kb mRNA in tissues such as testis, myometrium, placenta, and lung, and the protein product (relative molecular mass, 190,000) shows sequence-specific DNA binding. The GLI3 gene is mapped on 7p14.1. GLI3 is homologous to the Drosophila cubitus interruptus (ci) gene product (Ci), which regulates the Patched (pct), gooseberry (gsb), and decapentaplegic (dpp) genes. PKA-dependent processing of vertebrate GLI3 in developing limb generates a potent repressor in a manner antagonized by apparent long-range signaling from posteriorly localized Sonic hedgehog protein. The high relative abundance and potency of GLI3 repressor suggested specialization of GLI3 and its products for negative hedgehog pathway regulation.Coimmunoprecipitation and immunoblot studies showed that GLI3 protein is polyubiquitinated and that its processing depends on proteasome activity. The findings suggested that BTRC is required for GLI3 processing.

Transcriptional activator GLI3;GLI3 form of 190 kDa;GLI3-190;GLI3 full length protein;GLI3FL;Transcriptional repressor GLI3R;GLI3 C-terminally truncated form;GLI3 form of 83 kDa;GLI3-83;GLI3;

A synthetic peptide corresponding to a sequence at the N-terminus of human Gli3, different from the related rat sequence by two amino acids, and from the related mouse sequence by one amino acid.

Has a dual function as a transcriptional activator and a repressor of the sonic hedgehog (Shh) pathway, and plays a role in limb development. The full-length GLI3 form (GLI3FL) after phosphorylation and nuclear translocation, acts as an activator (GLI3A) while GLI3R, its C-terminally truncated form, acts as a repressor. A proper balance between the GLI3 activator and the repressor GLI3R, rather than the repressor gradient itself or the activator/repressor ratio gradient, specifies limb digit number and identity. In concert with TRPS1, plays a role in regulating the size of the zone of distal chondrocytes, in restricting the zone of PTHLH expression in distal cells and in activating chondrocyte proliferation. Binds to the minimal GLI-consensus sequence 5'-GGGTGGTC-3'. .

1. Barna, M., Pandolfi, P. P., Niswander, L. Gli3 and Plzf cooperate in proximal limb patterning at early stages of limb development. (Letter) Nature 436: 277-281, 2005. 2. Bose, J., Grotewold, L., Ruther, U. Pallister-Hall syndrome phenotype in mice mutant for Gli3. Hum. Molec. Genet. 11: 1129-1135, 2002. 3. Craig, D. W., Itty, A., Panganiban, C., Szelinger, S., Kruer, M. C., Sekar, A., Reiman, D., Narayanan, V., Stephan, D. A., Kerrigan, J. F.Identification of somatic chromosomal abnormalities in hypothalamic hamartoma tissue at the GLI3 locus. Am. J. Hum. Genet. 82: 366-374, 2008.

Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Nucleus. Cytoplasm. Cell projection, cilium. GLI3FL is localized predominantly in the cytoplasm while GLI3R resides mainly in the nucleus. Ciliary accumulation requires the presence of KIF7 and SMO. Translocation to the nucleus is promoted by interaction with ZIC1.