Anti-FHL 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. It is mapped to Xq26.3. 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. Morgan MJ, Madgwick AJ (Aug 1996) . Slim defines a novel family of LIM-proteins expressed in skeletal muscle. Biochemical and Biophysical Research Communications. 225 (2) : 632–8. 2. Lee SM, Tsui SK, Chan KK, Garcia-Barcelo M, Waye MM, Fung KP, Liew CC, Lee CY (Aug 1998) . Chromosomal mapping, tissue distribution and cDNA sequence of four-and-a-half LIM domain protein 1 (FHL1) . Gene. 216 (1) : 163–70. 3. Windpassinger C, Schoser B, Straub V, Hochmeister S, Noor A, Lohberger B, Farra N, Petek E, Schwarzbraun T, Ofner L, Löscher WN, Wagner K, Lochmüller H, Vincent JB, Quasthoff S (Jan 2008) . An X-linked myopathy with postural muscle atrophy and generalized hypertrophy, termed XMPMA, is caused by mutations in FHL1. American Journal of Human Genetics. 82 (1) : 88–99. doi:10.1016/j.ajhg.2007.09.004.