Anti-FHL1 Antibody Picoband® Fluoro647 Conjugated

Four and a half LIM domains protein 1 is a protein that in humans is encoded by the FHL1 gene. This gene encodes a member of the four-and-a-half-LIM-only protein family. Family members contain two highly conserved, tandemly arranged, zinc finger domains with four highly conserved cysteines binding a zinc atom in each zinc finger. Expression of these family members occurs in a cell- and tissue-specific mode and these proteins are involved in many cellular processes. Mutations in this gene have been found in patients with Emery-Dreifuss muscular dystrophy. Multiple alternately spliced transcript variants which encode different protein isoforms have been described.

Isoform 1 seems to be most abundant in each tissue and isoform 2 much less abundant. Isoform 1 is highly expressed in skeletal muscle and lung, and to a lesser extent in heart, brain and kidney. Isoform 2 was found in brain, lung kidney and genital organs.

May have an involvement in muscle development or hypertrophy. Isoform 2 binds to RBP-J and plays a negative regulatory role in the RBP-J-mediated transcription in mammalian systems.

1. Brown, S., McGrath, M. J., Ooms, L. M., Gurung, R., Maimone, M. M., Mitchell, C. A. Characterization of two isoforms of the skeletal muscle LIM protein 1, SLIM1: localization of SLIM1 at focal adhesions and the isoform slimmer in the nucleus of myoblasts and cytoplasm of myotubes suggests distinct roles in the cytoskeleton and in nuclear-cytoplasmic communication. J. Biol. Chem. 274: 27083-27091, 1999. 2. Emmanuele, V., Kubota, A., Garcia-Diaz, B., Garone, C., Akman, H. O., Sanchez-Gutierrez, D., Escudero, L. M., Kariya, S., Homma, S., Tanji, K., Quinzii, C. M., Hirano, M. Fhl1 W122S causes loss of protein function and late-onset mild myopathy. Hum. Molec. Genet. 24: 714-726, 2015. 3. Fimia, G. M., De Cesare, D., Sassone-Corsi, P. A family of LIM-only transcriptional coactivators: tissue-specific expression and selective activation of CREB and CREM. Molec. Cell. Biol. 20: 8613-8622, 2000.