FHL1

Summary

Gene Symbol: FHL1
Description: four and a half LIM domains 1
Alias: FCMSU, FHL-1, FHL1A, FHL1B, FLH1A, KYOT, RBMX1A, RBMX1B, SLIM, SLIM-1, SLIM1, SLIMMER, XMPMA, four and a half LIM domains protein 1, LIM protein SLIMMER, four-and-a-half Lin11, Isl-1 and Mec-3 domains 1, skeletal muscle LIM-protein 1
Species: human
Products:     FHL1

Top Publications

  1. Schessl J, Zou Y, McGrath M, Cowling B, Maiti B, Chin S, et al. Proteomic identification of FHL1 as the protein mutated in human reducing body myopathy. J Clin Invest. 2008;118:904-12 pubmed publisher
    ..The most prominent component of the inclusions was the Xq26.3-encoded four and a half LIM domain 1 (FHL1) protein, expressed predominantly in skeletal but also in cardiac muscle...
  2. Morgan M, Madgwick A, Charleston B, Pell J, Loughna P. The developmental regulation of a novel muscle LIM-protein. Biochem Biophys Res Commun. 1995;212:840-6 pubmed
    ..This period corresponds to the period of greatest muscle fibre hypertrophy and suggests a role for this homologue in either the elaboration of muscle fibre matrix anchorage or the regulation of muscle fibre hypertrophy itself. ..
  3. Morgan M, Madgwick A. The LIM proteins FHL1 and FHL3 are expressed differently in skeletal muscle. Biochem Biophys Res Commun. 1999;255:245-50 pubmed
    ..The distribution of FHL mRNA expression within a variety of murine tissues is complex. Both FHL1 and FHL3 were expressed in a number of skeletal muscles while FHL2 was expressed at high levels in cardiac muscle...
  4. Ding L, Wang Z, Yan J, Yang X, Liu A, Qiu W, et al. Human four-and-a-half LIM family members suppress tumor cell growth through a TGF-beta-like signaling pathway. J Clin Invest. 2009;119:349-61 pubmed publisher
    ..The exact functions of each FHL protein in cancer development and progression remain unknown. Here we report that FHL1, FHL2, and FHL3 physically and functionally interact with Smad2, Smad3, and Smad4, important regulators of cancer ..
  5. Brown S, McGrath M, Ooms L, Gurung R, Maimone M, Mitchell C. 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 nu. J Biol Chem. 1999;274:27083-91 pubmed
    We have cloned and characterized a novel isoform of the skeletal muscle LIM protein 1 (SLIM1), designated SLIMMER. SLIM1 contains an N-terminal single zinc finger followed by four LIM domains...
  6. Quinzii C, Vu T, Min K, Tanji K, Barral S, Grewal R, et al. X-linked dominant scapuloperoneal myopathy is due to a mutation in the gene encoding four-and-a-half-LIM protein 1. Am J Hum Genet. 2008;82:208-13 pubmed publisher
    ..365G-->C) in the FHL1 gene encoding four-and-a-half-LIM protein 1 (FHL1)...
  7. Ng E, Lee S, Li H, Ngai S, Tsui S, Waye M, et al. Characterization of tissue-specific LIM domain protein (FHL1C) which is an alternatively spliced isoform of a human LIM-only protein (FHL1). J Cell Biochem. 2001;82:1-10 pubmed
    ..Unlike FHL1B, which is another FHL1 isoform recently reported by our group and localized predominantly in the nucleus [Lee et ..
  8. Friedrich F, Wilding B, Reischmann S, Crocini C, Lang P, Charron P, et al. Evidence for FHL1 as a novel disease gene for isolated hypertrophic cardiomyopathy. Hum Mol Genet. 2012;21:3237-54 pubmed publisher
    ..We hypothesized that FHL1, encoding four-and-a-half-LIM domains 1, could be another disease gene since it has been shown to cause distinct ..
  9. Chen D, Raskind W, Parson W, Sonnen J, Vu T, Zheng Y, et al. A novel mutation in FHL1 in a family with X-linked scapuloperoneal myopathy: phenotypic spectrum and structural study of FHL1 mutations. J Neurol Sci. 2010;296:22-9 pubmed publisher
    An X-linked myopathy was recently associated with mutations in the four-and-a-half-LIM domains 1 (FHL1) gene...

More Information

Publications90

  1. Knoblauch H, Geier C, Adams S, Budde B, Rudolph A, Zacharias U, et al. Contractures and hypertrophic cardiomyopathy in a novel FHL1 mutation. Ann Neurol. 2010;67:136-40 pubmed publisher
    ..We used microsatellite markers and found linkage to a locus at Xq26-28, a region harboring the FHL1 gene...
  2. Kwapiszewska G, Wygrecka M, Marsh L, Schmitt S, Trösser R, Wilhelm J, et al. Fhl-1, a new key protein in pulmonary hypertension. Circulation. 2008;118:1183-94 pubmed publisher
    ..Coimmunoprecipitation experiments identified Talin1 as a new interacting partner of Fhl-1. Protein screening identified Fhl-1 as a novel protein regulated in various forms of PH, including idiopathic pulmonary arterial hypertension. ..
  3. Cowling B, McGrath M, Nguyen M, Cottle D, Kee A, Brown S, et al. Identification of FHL1 as a regulator of skeletal muscle mass: implications for human myopathy. J Cell Biol. 2008;183:1033-48 pubmed publisher
    ..Four-and-a-half LIM protein 1 (FHL1) is mutated in several human myopathies, including reducing-body myopathy (RBM)...
  4. Schessl J, Taratuto A, Sewry C, Battini R, Chin S, Maiti B, et al. Clinical, histological and genetic characterization of reducing body myopathy caused by mutations in FHL1. Brain. 2009;132:452-64 pubmed publisher
    We recently identified the X-chromosomal four and a half LIM domain gene FHL1 as the causative gene for reducing body myopathy, a disorder characterized by progressive weakness and intracytoplasmic aggregates in muscle that exert reducing ..
  5. Schoser B, Goebel H, Janisch I, Quasthoff S, Rother J, Bergmann M, et al. Consequences of mutations within the C terminus of the FHL1 gene. Neurology. 2009;73:543-51 pubmed publisher
    ..myopathy characterized by postural muscle atrophy with rigid spine syndrome with pseudoathleticism/hypertrophy (XMPMA), reducing body myopathy (RBM), and scapuloperoneal myopathy...
  6. Gueneau L, Bertrand A, Jais J, Salih M, Stojkovic T, Wehnert M, et al. Mutations of the FHL1 gene cause Emery-Dreifuss muscular dystrophy. Am J Hum Genet. 2009;85:338-53 pubmed publisher
    ..By whole-genome scan, we identified linkage to the Xq26.3 locus containing the FHL1 gene in three informative families belonging to our EMD- and LMNA-negative cohort...
  7. Asada K, Ando T, Niwa T, Nanjo S, Watanabe N, Okochi Takada E, et al. FHL1 on chromosome X is a single-hit gastrointestinal tumor-suppressor gene and contributes to the formation of an epigenetic field defect. Oncogene. 2013;32:2140-9 pubmed publisher
    ..Among them, FHL1 was frequently methylation-silenced in gastric and colon cancer cell lines, and methylated in primary gastric (21/..
  8. Weng J, Liao M, Zou S, Bao J, Zhou J, Qu L, et al. Downregulation of FHL1 expression in thoracic aortic dissection: implications in aortic wall remodeling and pathogenesis of thoracic aortic dissection. Ann Vasc Surg. 2011;25:240-7 pubmed publisher
    To investigate the potential role of four and a half Lin-11, Isl-1, and Mec-3 (FHL1) protein in the pathogenesis of thoracic aortic dissection (TAD)...
  9. Cowling B, Cottle D, Wilding B, D Arcy C, Mitchell C, McGrath M. Four and a half LIM protein 1 gene mutations cause four distinct human myopathies: a comprehensive review of the clinical, histological and pathological features. Neuromuscul Disord. 2011;21:237-51 pubmed publisher
    Mutations in the four and a half LIM protein 1 (FHL1) gene were recently identified as the cause of four distinct skeletal muscle diseases...
  10. Niu C, Liang C, Guo J, Cheng L, Zhang H, Qin X, et al. Downregulation and growth inhibitory role of FHL1 in lung cancer. Int J Cancer. 2012;130:2549-56 pubmed publisher
    Four and a half Lin-11, Isl-1, Mac-3 (LIM) protein 1 (FHL1) has been linked to carcinogenesis. However, the role of FHL1 in lung cancer remains unclear and the detailed mechanism underlying its tumor suppressive role is poorly understood...
  11. Xu Y, Liu Z, Guo K. Expression of FHL1 in gastric cancer tissue and its correlation with the invasion and metastasis of gastric cancer. Mol Cell Biochem. 2012;363:93-9 pubmed publisher
    This study was performed to analyze the expression of four and a half LIM domains 1 (FHL1) in gastric carcinoma tissue and its correlation with the clinicopathological characteristics of gastric cancer...
  12. McGrath M, Mitchell C, Coghill I, Robinson P, Brown S. Skeletal muscle LIM protein 1 (SLIM1/FHL1) induces alpha 5 beta 1-integrin-dependent myocyte elongation. Am J Physiol Cell Physiol. 2003;285:C1513-26 pubmed
    b>Skeletal muscle LIM protein 1 (SLIM1/FHL1) contains four and a half LIM domains and is highly expressed in skeletal and cardiac muscle...
  13. Qin H, Wang J, Liang Y, Taniguchi Y, Tanigaki K, Han H. RING1 inhibits transactivation of RBP-J by Notch through interaction with LIM protein KyoT2. Nucleic Acids Res. 2004;32:1492-501 pubmed
    ..Taken together, our data suggested that there might be at least two ways of the KyoT2-mediated suppression of RBP-J, namely competition for binding sites with transactivators, and recruitment of suppressors such as RING1. ..
  14. Lee S, Tsui S, Chan K, Garcia Barcelo M, Waye M, Fung K, et al. Chromosomal mapping, tissue distribution and cDNA sequence of four-and-a-half LIM domain protein 1 (FHL1). Gene. 1998;216:163-70 pubmed
    ..of LIM domain and an extra zinc finger and this putative protein is named four-and-a-half LIM domain protein 1 (FHL1). FHL1 is unique when compared with other LIM-only proteins because it possesses an odd number of zinc fingers...
  15. Windpassinger C, Schoser B, Straub V, Hochmeister S, Noor A, Lohberger B, et al. An X-linked myopathy with postural muscle atrophy and generalized hypertrophy, termed XMPMA, is caused by mutations in FHL1. Am J Hum Genet. 2008;82:88-99 pubmed publisher
    ..In summary, we have to our knowledge characterized a new disorder, X-linked myopathy with postural muscle atrophy (XMPMA), and identified FHL1 as the causative gene...
  16. Lee S, Li H, Ng E, Or S, Chan K, Kotaka M, et al. Characterization of a brain-specific nuclear LIM domain protein (FHL1B) which is an alternatively spliced variant of FHL1. Gene. 1999;237:253-63 pubmed
    ..This gene is located at chromosome Xq27 and the spliced variant is named FHL1B. The ORF of FHL1B cDNA codes for a LIM-only protein that possesses a zinc finger and three tandem repeats of LIM ..
  17. McGrath M, Cottle D, Nguyen M, Dyson J, Coghill I, Robinson P, et al. Four and a half LIM protein 1 binds myosin-binding protein C and regulates myosin filament formation and sarcomere assembly. J Biol Chem. 2006;281:7666-83 pubmed
    Four and a half LIM protein 1 (FHL1/SLIM1) is highly expressed in skeletal and cardiac muscle; however, the function of FHL1 remains unknown...
  18. Greene W, Baker E, Rabbitts T, Kees U. Genomic structure, tissue expression and chromosomal location of the LIM-only gene, SLIM1. Gene. 1999;232:203-7 pubmed
    Human SLIM1 is a recently described gene of the LIM-only class encoding four and a half tandemly repeated LIM domains...
  19. Qin H, Du D, Zhu Y, Li J, Feng L, Liang Y, et al. The PcG protein HPC2 inhibits RBP-J-mediated transcription by interacting with LIM protein KyoT2. FEBS Lett. 2005;579:1220-6 pubmed
    ..Taken together, our results suggested that KyoT2 might inhibit the RBP-J-mediated transactivation through NIC by recruiting co-suppressors such as HPC2. ..
  20. Yang Z, Browning C, Hallaq H, Yermalitskaya L, Esker J, Hall M, et al. Four and a half LIM protein 1: a partner for KCNA5 in human atrium. Cardiovasc Res. 2008;78:449-57 pubmed publisher
    ..spectrometry-based methods, the scaffolding protein four and a half LIM (for Lin-11, Isl-1, and Mec3) protein 1 (FHL1) was identified as a potential protein partner for KCNA5...
  21. Matsumoto M, Kawakami K, Enokida H, Toki K, Matsuda R, Chiyomaru T, et al. CpG hypermethylation of human four-and-a-half LIM domains 1 contributes to migration and invasion activity of human bladder cancer. Int J Mol Med. 2010;26:241-7 pubmed
    ..We focused on the human four-and-a-half LIM domains 1 (FHL1) gene which was selected on the basis of previous microarray data analysis...
  22. Wilding B, McGrath M, Bonne G, Mitchell C. FHL1 mutants that cause clinically distinct human myopathies form protein aggregates and impair myoblast differentiation. J Cell Sci. 2014;127:2269-81 pubmed publisher
    ..reducing body myopathy (RBM), scapuloperoneal myopathy (SPM) and X-linked myopathy with postural muscle atrophy (XMPMA). The molecular mechanisms underlying the pathogenesis of FHL1 myopathies are unknown...
  23. Luo S, Poltermann S, Kunert A, Rupp S, Zipfel P. Immune evasion of the human pathogenic yeast Candida albicans: Pra1 is a Factor H, FHL-1 and plasminogen binding surface protein. Mol Immunol. 2009;47:541-50 pubmed publisher
    ..As a released protein, Pra1 enhances complement control in direct vicinity of the yeast and thus generates an additional protective layer which controls host complement attack. ..
  24. Zhang F, Feng F, Yang P, Li Z, You J, Xie W, et al. Four-and-a-half-LIM protein 1 down-regulates estrogen receptor ? activity through repression of AKT phosphorylation in human breast cancer cell. Int J Biochem Cell Biol. 2012;44:320-6 pubmed publisher
    ..By establishing a linkage role of the FHL-1 between the estrogen ER? signaling pathway and IGF/PI3K/AKT signaling pathway, this study identifies that FHL-1 proteins may be a useful molecular target for human breast cancer therapy. ..
  25. Fan Z, Xu X, Zhang X, Wang T, Han B, Liang Y, et al. [FHL1 knockdown mediated by lentiviral shRNA promotes the growth of HeLa and HepG2 cells]. Xi Bao Yu Fen Zi Mian Yi Xue Za Zhi. 2015;31:879-83 pubmed
    To investigate the effect of four-and-a-half LIM domain 1 (FHL1) knockdown by lentiviral-mediated shRNA on the growth of HeLa and HepG2 cells. pLenti-H1 FHL1 shRNA was cloned, and then transfered into HEK293T cells...
  26. Clark S, Schmidt C, White A, Hakobyan S, Morgan B, Bishop P. Identification of factor H-like protein 1 as the predominant complement regulator in Bruch's membrane: implications for age-related macular degeneration. J Immunol. 2014;193:4962-70 pubmed publisher
    ..Our results identify a novel mechanism of complement regulation in the human eye, which highlights potential new avenues for therapeutic strategies. ..
  27. Wu S, Shih L, Lee J, Shen Y, Lee H. Estrogen enhances activity of Wnt signaling during osteogenesis by inducing Fhl1 expression. J Cell Biochem. 2015;116:1419-30 pubmed publisher
    ..In this study, we show that the Wnt agonist, LiCl, induced Fhl1 gene expression, which substantially enhanced osteoblast differentiation...
  28. Xu J, Cui J, del Campo A, Shin C. Four and a Half LIM Domains 1b (Fhl1b) Is Essential for Regulating the Liver versus Pancreas Fate Decision and for β-Cell Regeneration. PLoS Genet. 2016;12:e1005831 pubmed publisher
    ..We have identified four and a half LIM domains 1b (fhl1b), which is primarily expressed in the prospective liver anlage, as a novel target of Bmp2b signaling...
  29. Malfatti E, Olivé M, Taratuto A, Richard P, Brochier G, Bitoun M, et al. Skeletal muscle biopsy analysis in reducing body myopathy and other FHL1-related disorders. J Neuropathol Exp Neurol. 2013;72:833-45 pubmed publisher
    b>FHL1 mutations have been associated with various disorders that include reducing body myopathy (RBM), Emery-Dreifuss-like muscular dystrophy, isolated hypertrophic cardiomyopathy, and some overlapping conditions...
  30. Wang X, Wei X, Yuan Y, Sun Q, Zhan J, Zhang J, et al. Src-mediated phosphorylation converts FHL1 from tumor suppressor to tumor promoter. J Cell Biol. 2018;217:1335-1351 pubmed publisher
    b>FHL1 has been recognized for a long time as a tumor suppressor protein that associates with both the actin cytoskeleton and the transcriptional machinery...
  31. Waddell L, Tran J, Zheng X, Bönnemann C, Hu Y, Evesson F, et al. A study of FHL1, BAG3, MATR3, PTRF and TCAP in Australian muscular dystrophy patients. Neuromuscul Disord. 2011;21:776-81 pubmed publisher
    b>FHL1, BAG3, MATR3 and PTRF are recently identified myopathy genes associated with phenotypes that overlap muscular dystrophy. TCAP is a rare reported cause of muscular dystrophy not routinely screened in most centres...
  32. Tai A, Kamei Y, Mukai Y. The forkhead-like transcription factor (Fhl1p) maintains yeast replicative lifespan by regulating ribonucleotide reductase 1 (RNR1) gene transcription. Biochem Biophys Res Commun. 2017;488:218-223 pubmed publisher
    ..As previously reported, the forkhead-like transcription factor (FHL1) gene was required for yeast replicative lifespan and cell proliferation...
  33. Le Thanh P, Meinke P, Korfali N, Srsen V, Robson M, Wehnert M, et al. Immunohistochemistry on a panel of Emery-Dreifuss muscular dystrophy samples reveals nuclear envelope proteins as inconsistent markers for pathology. Neuromuscul Disord. 2017;27:338-351 pubmed publisher
    ..a panel of nine Emery-Dreifuss muscular dystrophy patients (4 male, 5 female) including those carrying emerin and FHL1 (X-linked) and several lamin A (autosomal dominant) mutations were stained for the proteins linked to the disorder...
  34. Koike K, Kasamatsu A, Iyoda M, Saito Y, Kouzu Y, Koike H, et al. High prevalence of epigenetic inactivation of the human four and a half LIM domains 1 gene in human oral cancer. Int J Oncol. 2013;42:141-50 pubmed publisher
    The four and a half LIM domains 1 (FHL1) gene has been related to carcinogenesis...
  35. Cao C, Mok S, Cheng V, Tsui S. The FHL2 regulation in the transcriptional circuitry of human cancers. Gene. 2015;572:1-7 pubmed publisher
    ..Five members have been categorized into the FHL subfamily, which are FHL1, FHL2, FHL3, FHL4 and activator of CREM in testis (ACT) in human...
  36. Hubbi M, Gilkes D, Baek J, Semenza G. Four-and-a-half LIM domain proteins inhibit transactivation by hypoxia-inducible factor 1. J Biol Chem. 2012;287:6139-49 pubmed publisher
    ..Here we describe functional interactions between the FHL proteins and HIF-1. FHL1-3 inhibit HIF-1 transcriptional activity and HIF-1? transactivation domain function by oxygen-independent ..
  37. Morgan M, Madgwick A. Slim defines a novel family of LIM-proteins expressed in skeletal muscle. Biochem Biophys Res Commun. 1996;225:632-8 pubmed
    We have assembled the complete protein sequence of the skeletal muscle LIM-protein SLIM by aligning overlapping cDNA sequences...
  38. Pataki E, Weisman R, Sipiczki M, Miklos I. fhl1 gene of the fission yeast regulates transcription of meiotic genes and nitrogen starvation response, downstream of the TORC1 pathway. Curr Genet. 2017;63:91-101 pubmed publisher
    ..Our previous data revealed that the fhl1 fork-head type transcription factor of the fission yeast could be involved in sporulation, which was typically ..
  39. Dahlquist K, Fitzpatrick B, Camacho E, Entzminger S, Wanner N. Parameter Estimation for Gene Regulatory Networks from Microarray Data: Cold Shock Response in Saccharomyces cerevisiae. Bull Math Biol. 2015;77:1457-92 pubmed publisher
    ..The modeling results newly suggest that Rap1, Fhl1, Msn4, Rph1, and Hsf1 play an important role in regulating the early response to cold shock in yeast...
  40. Ren W, Lian P, Cheng L, Du P, Guan X, Wang H, et al. FHL1 inhibits the growth of tongue squamous cell carcinoma cells via G1/S cell cycle arrest. Mol Med Rep. 2015;12:3958-3964 pubmed publisher
    Four and a half LIM protein 1 (FHL1) has been characterized as a tumor suppressor in various types of tumor...
  41. Schreckenbach T, Henn W, Kress W, Roos A, Maschke M, Feiden W, et al. Novel FHL1 mutation in a family with reducing body myopathy. Muscle Nerve. 2013;47:127-34 pubmed publisher
    ..It is caused by mutations in the FHL1 gene, which encodes the four-and-a-half LIM domain 1 protein (FHL1)...
  42. Albrecht I, Wick C, Hallgren Ã, Tjärnlund A, Nagaraju K, Andrade F, et al. Development of autoantibodies against muscle-specific FHL1 in severe inflammatory myopathies. J Clin Invest. 2015;125:4612-24 pubmed publisher
    Mutations of the gene encoding four-and-a-half LIM domain 1 (FHL1) are the causative factor of several X-linked hereditary myopathies that are collectively termed FHL1-related myopathies...
  43. Neve A, Trüb J, Saxena S, Schümperli D. Central and peripheral defects in motor units of the diaphragm of spinal muscular atrophy mice. Mol Cell Neurosci. 2016;70:30-41 pubmed publisher
    ..1 (AIFM1), a mitochondrial flavoprotein that initiates apoptosis in a caspase-independent pathway, and four and a half Lim domain protein 1 (FHL1), a regulator of skeletal muscle mass that has been implicated in several myopathies.
  44. Tanaka T, Soriano M, Grusby M. SLIM is a nuclear ubiquitin E3 ligase that negatively regulates STAT signaling. Immunity. 2005;22:729-36 pubmed
    ..We show that SLIM is an ubiquitin E3 ligase that acts on STAT proteins to cause their proteosome-mediated degradation and enhance ..
  45. Koprivova A, Kopriva S. Hormonal control of sulfate uptake and assimilation. Plant Mol Biol. 2016;91:617-27 pubmed publisher
    ..assimilation is the fact that the main regulator of the response to sulfate starvation, SULFATE LIMITATION1 (SLIM1) belongs to the family of ethylene related transcription factors...
  46. Kim M, Hahn J. Role of CK2-dependent phosphorylation of Ifh1 and Crf1 in transcriptional regulation of ribosomal protein genes in Saccharomyces cerevisiae. Biochim Biophys Acta. 2016;1859:1004-13 pubmed publisher
    In Saccharomyces cerevisiae, Fhl1 is involved in the regulation of ribosomal protein (RP) genes through interaction with either its coactivator Ifh1 or corepressor Crf1, depending on nutrient conditions...
  47. Wang J, Huang F, Huang J, Kong J, Liu S, Jin J. Epigenetic analysis of FHL1 tumor suppressor gene in human liver cancer. Oncol Lett. 2017;14:6109-6116 pubmed publisher
    Liver cancer is one of the most common types of cancer among human malignancies. Four and a half LIM domains 1 (FHL1), as a tumor suppressor gene, is frequently downregulated in multiple types of human cancer...
  48. Willis T, Wood C, Hudson J, Polvikoski T, Barresi R, Lochmuller H, et al. Muscle hypertrophy as the presenting sign in a patient with a complete FHL1 deletion. Clin Genet. 2016;90:166-70 pubmed publisher
    Four and a half LIM protein 1 (FHL1/SLIM1) has recently been identified as the causative gene mutated in four distinct diseases affecting skeletal muscle that have overlapping features, including reducing body myopathy, X-linked myopathy,..
  49. Xu X, Fan Z, Liang C, Li L, Wang L, Liang Y, et al. A signature motif in LIM proteins mediates binding to checkpoint proteins and increases tumour radiosensitivity. Nat Commun. 2017;8:14059 pubmed publisher
    ..Here, we show that LIM domain-containing proteins, such as FHL1, increase inhibitory CDC25 phosphorylation by forming a complex with CHK2 and CDC25, and sequester CDC25 in the ..
  50. Philippar U, Schratt G, Dieterich C, Müller J, Galgoczy P, Engel F, et al. The SRF target gene Fhl2 antagonizes RhoA/MAL-dependent activation of SRF. Mol Cell. 2004;16:867-80 pubmed
    ..Our findings identify an autoregulatory mechanism to selectively regulate subsets of RhoA-activated SRF target genes. ..
  51. Cao W, Liu J, Xia R, Lin L, Wang X, Xiao M, et al. X-linked FHL1 as a novel therapeutic target for head and neck squamous cell carcinoma. Oncotarget. 2016;7:14537-50 pubmed publisher
    ..of gene transcriptional profiling, we identified downregulation of X-linked four-and-a-half LIM domains protein 1 (FHL1) gene in head and neck squamous cell carcinoma (HNSCC)...
  52. Christodoulou D, Wakimoto H, Onoue K, Eminaga S, Gorham J, DePalma S, et al. 5'RNA-Seq identifies Fhl1 as a genetic modifier in cardiomyopathy. J Clin Invest. 2014;124:1364-70 pubmed publisher
    ..92 genes had altered start-site usage in a mouse model of HCM, including four-and-a-half LIM domains protein 1 (Fhl1)...
  53. Wong C, Fung Y, Ng E, Lee S, Waye M, Tsui S. LIM domain protein FHL1B interacts with PP2A catalytic ? subunit--a novel cell cycle regulatory pathway. FEBS Lett. 2010;584:4511-6 pubmed publisher
    Four-and-a-half LIM domain protein 1B (FHL1B) is an alternatively-spliced isoform of FHL1...
  54. Ravel Chapuis A, Belanger G, Cote J, Michel R, Jasmin B. Misregulation of calcium-handling proteins promotes hyperactivation of calcineurin-NFAT signaling in skeletal muscle of DM1 mice. Hum Mol Genet. 2017;26:2192-2206 pubmed publisher
    ..Coherent with this, we found that activators of the CnA pathway (MLP, FHL1) are also elevated...
  55. San Román I, Navarro M, Martinez F, Albert L, Polo L, Guardiola J, et al. Unclassifiable arrhythmic cardiomyopathy associated with Emery-Dreifuss caused by a mutation in FHL1. Clin Genet. 2016;90:171-6 pubmed publisher
    ..We characterize clinically a large family with a mutation in FHL1 gene (p.Cys255Ser). Penetrance was 44%, 100% for males and 18% for females. The heart was the main organ involved...
  56. Tani S, Taniwaki M, Taniguchi Y, Minoguchi S, Kuroda K, Han H, et al. Chromosomal mapping of two RBP-J-related genes: Kyo-T and RBP-L. J Hum Genet. 1999;44:73-5 pubmed
    ..RBP-L has a highly homologous region with RBP-J but the function of RBP-L is unknown. Fluorescence in situ hybridization analysis of human metaphase chromosomes localized Kyo-T and RBP-L to Xq26 and 20q12-13.1, respectively. ..
  57. D Avila F, Meregalli M, Lupoli S, Barcella M, Orro A, De Santis F, et al. Exome sequencing identifies variants in two genes encoding the LIM-proteins NRAP and FHL1 in an Italian patient with BAG3 myofibrillar myopathy. J Muscle Res Cell Motil. 2016;37:101-15 pubmed publisher
    ..In the patient, we found that the BAG3 mutation is associated with variants in the NRAP and FHL1 genes that encode muscle-specific, LIM domain containing proteins...
  58. He H, Hu Z, Tserennadmid S, Chen S, Liu X. Novel Muscle-Specific Genes TCAP, TNNI1, and FHL1 in Cattle: SNVs, Linkage Disequilibrium, Combined Genotypes, Association Analysis of Growth Performance, and Carcass Quality Traits and Expression Studies. Anim Biotechnol. 2017;:1-10 pubmed publisher
    TCAP, TNNI1, and FHL1 regulate muscle growth and development...
  59. Topçuoğlu B, Meydan C, Orellana R, Holden J. Formate hydrogenlyase and formate secretion ameliorate H2 inhibition in the hyperthermophilic archaeon Thermococcus paralvinellae. Environ Microbiol. 2017;: pubmed publisher
    ..In contrast, expression of the formate hydrogenlyase 1 (fhl1) operon increased with added H2 ...
  60. Tiffin H, Jenkins Z, Gray M, Cameron Christie S, Eaton J, Aftimos S, et al. Dysregulation of FHL1 spliceforms due to an indel mutation produces an Emery-Dreifuss muscular dystrophy plus phenotype. Neurogenetics. 2013;14:113-21 pubmed publisher
    ..The more common X-linked recessive form of EDMD is caused by mutations in either EMD (encoding emerin) or FHL1 (encoding four and a half LIM domains 1), while mutations in LMNA (encoding lamin A/C), SYNE1 (encoding nesprin-1) ..
  61. Atay S, Wilkey D, Milhem M, Merchant M, Godwin A. Insights into the Proteome of Gastrointestinal Stromal Tumors-Derived Exosomes Reveals New Potential Diagnostic Biomarkers. Mol Cell Proteomics. 2018;17:495-515 pubmed publisher
    ..g. DPP4, FHL1, CDH11, and KCTD12)...
  62. Pen A, Nyegaard M, Fang M, Jiang H, Christensen R, Mølgaard H, et al. A novel single nucleotide splice site mutation in FHL1 confirms an Emery-Dreifuss plus phenotype with pulmonary artery hypoplasia and facial dysmorphology. Eur J Med Genet. 2015;58:222-9 pubmed publisher
    ..FHL1 expresses three main splice variants, known as FHL1A, FHL1B and FHL1C...
  63. Domenighetti A, Chu P, Wu T, Sheikh F, Gokhin D, Guo L, et al. Loss of FHL1 induces an age-dependent skeletal muscle myopathy associated with myofibrillar and intermyofibrillar disorganization in mice. Hum Mol Genet. 2014;23:209-25 pubmed publisher
    ..early muscle fiber differentiation and maturation defects, which could be rescued by re-expression of the FHL1A isoform, highlighting that FHL1A is necessary for proper muscle fiber differentiation and maturation in vitro...
  64. Hartmannova H, Kubanek M, Sramko M, Piherová L, Noskova L, Hodanova K, et al. Isolated X-linked hypertrophic cardiomyopathy caused by a novel mutation of the four-and-a-half LIM domain 1 gene. Circ Cardiovasc Genet. 2013;6:543-51 pubmed publisher
    ..A novel frameshift mutation of four-and-a-half LIM domain 1 gene (FHL1) (c.599_600insT; p.F200fs32X) was detected in these individuals...
  65. Wang L, Gu H, Fan Y, Zhang Y, Wu D, Miao J, et al. Up-regulated FHL1 expression maybe involved in the prognosis of Hirschsprung's disease. Int J Med Sci. 2014;11:262-7 pubmed publisher
    ..Inhibition of four-and-a-half LIM protein-1 (Fhl1) expression by siRNA significantly decreases pulmonary artery SMCs migration and proliferation...
  66. Wang Y, Fu J, Jiang M, Zhang X, Cheng L, Xu X, et al. MiR-410 is overexpressed in liver and colorectal tumors and enhances tumor cell growth by silencing FHL1 via a direct/indirect mechanism. PLoS ONE. 2014;9:e108708 pubmed publisher
    b>FHL1 is an important tumor-suppressor that is downregulated in multiple tumors by unknown mechanisms. We demonstrated that miR-410 specifically targets the 3'UTR of FHL1...
  67. Lin J, Ding L, Jin R, Zhang H, Cheng L, Qin X, et al. Four and a half LIM domains 1 (FHL1) and receptor interacting protein of 140kDa (RIP140) interact and cooperate in estrogen signaling. Int J Biochem Cell Biol. 2009;41:1613-8 pubmed publisher
    Four and a half LIM domains 1 (FHL1) belongs to a family of LIM-only proteins that regulate gene transcription, cell proliferation, differentiation and apoptosis. However, the biological function of FHL1 remains largely unknown...
  68. Shalaby S, Hayashi Y, Nonaka I, Noguchi S, Nishino I. Novel FHL1 mutations in fatal and benign reducing body myopathy. Neurology. 2009;72:375-6 pubmed publisher
  69. Schessl J, Columbus A, Hu Y, Zou Y, Voit T, Goebel H, et al. Familial reducing body myopathy with cytoplasmic bodies and rigid spine revisited: identification of a second LIM domain mutation in FHL1. Neuropediatrics. 2010;41:43-6 pubmed publisher
    ..We recently identified the four and a half LIM domain gene FHL1 located on chromosome Xq26 as the causative gene for RBM...
  70. Sakashita K, Mimori K, Tanaka F, Kamohara Y, Inoue H, Sawada T, et al. Clinical significance of loss of Fhl1 expression in human gastric cancer. Ann Surg Oncol. 2008;15:2293-300 pubmed publisher
    Human four-and-a-half LIM domains 1 (Fhl1) gene has been reported to achieve cancer suppressive effects. The purpose of this study is to clarify clinical significance of Fhl1 expression in gastric cancer...
  71. Raskin A, Lange S, Banares K, Lyon R, Zieseniss A, Lee L, et al. A novel mechanism involving four-and-a-half LIM domain protein-1 and extracellular signal-regulated kinase-2 regulates titin phosphorylation and mechanics. J Biol Chem. 2012;287:29273-84 pubmed publisher
    ..complex at the cardiac-specific N2B region of titin that includes four-and-a-half LIM domain protein-1 (Fhl1) and components of the mitogen-activated protein signaling cascade, which impacted muscle compliance in Fhl1 knock-..
  72. Sarkozy A, Windpassinger C, Hudson J, Dougan C, Lecky B, Hilton Jones D, et al. Phenotypic heterogeneity in British patients with a founder mutation in the FHL1 gene. Eur J Hum Genet. 2011;19:1038-44 pubmed publisher
    Mutations in the four-and-a-half LIM domain 1 (FHL1) gene, which encodes a 280-amino-acid protein containing four LIM domains and a single zinc-finger domain in the N-terminal region, have been associated with a broad clinical spectrum of ..
  73. Sato K, Kimura M, Sugiyama K, Nishikawa M, Okano Y, Nagaoka H, et al. Four-and-a-half LIM Domains 1 (FHL1) Protein Interacts with the Rho Guanine Nucleotide Exchange Factor PLEKHG2/FLJ00018 and Regulates Cell Morphogenesis. J Biol Chem. 2016;291:25227-25238 pubmed
    ..Among the isoforms of FHL1 (i.e. FHL1A, FHL1B, and FHL1C), FHL1A and FHL1B interacted with PLEKHG2...
  74. Pillar N, Pleniceanu O, Fang M, Ziv L, Lahav E, Botchan S, et al. A rare variant in the FHL1 gene associated with X-linked recessive hypoparathyroidism. Hum Genet. 2017;136:835-845 pubmed publisher
    ..analysis coupling its action to known modulators of PTH function; (ii) observing strong expression of fhl1b in Corpuscles of Stannius, gland-like aggregates in zebrafish that function in calcium regulation similar to ..
  75. Wang L, Fu W, Li Z, Sun K. [Research of HOXD13 and FHL1 in idiopathic congenital talipes equinovarus]. Yi Chuan. 2008;30:46-50 pubmed
    To investigate the relationship of HOXD13 and FHL1 in idiopathic congenital talipes equinovarus(ICTEV), 84 samples from patients with ICTEV were used in the study...
  76. Ehsan M, Jiang H, L Thomson K, Gehmlich K. When signalling goes wrong: pathogenic variants in structural and signalling proteins causing cardiomyopathies. J Muscle Res Cell Motil. 2017;38:303-316 pubmed publisher
    ..Here, we focus on a subgroup of cardiomyopathy genes [TTN, FHL1, CSRP3, FLNC and PLN, coding for Titin, Four and a Half LIM domain 1, Muscle LIM Protein, Filamin C and ..
  77. Komagamine T, Kawai M, Kokubun N, Miyatake S, Ogata K, Hayashi Y, et al. Selective muscle involvement in a family affected by a second LIM domain mutation of fhl1: an imaging study using computed tomography. J Neurol Sci. 2012;318:163-7 pubmed publisher
    Mutations in the four-and-a-half LIM domains 1 gene (fhl1) are associated with various phenotypes of hereditary myopathies, including reducing body myopathy...
  78. Xue Y, Schoser B, Rao A, Quadrelli R, Vaglio A, Rupp V, et al. Exome Sequencing Identified a Splice Site Mutation in FHL1 that Causes Uruguay Syndrome, an X-Linked Disorder With Skeletal Muscle Hypertrophy and Premature Cardiac Death. Circ Cardiovasc Genet. 2016;9:130-5 pubmed publisher
    ..502-2A>G) in the FHL1 gene was highly suspicious among other candidate genes and variants. FHL1A is the predominant isoform of FHL1 in cardiac and skeletal muscle...