Identification of an FHL1 Protein Complex Containing Gamma-Actin and Non-Muscle Myosin IIB by Analysis of Protein-Protein Interactions

Wang, Lili; Jiang, Miao; Li, Lianyong; Wu, Di; Zhang, Yi; Peng, Zhaohong; Zhang, Lijun; Yuan, Zhengwei; Sun, Kun

doi:10.1371/journal.pone.0079551

Cited by 4 publications

(4 citation statements)

References 50 publications

(57 reference statements)

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“…The ACTG1-encoded protein (cytoplasmic γ-1-actin), an actin isoform, is involved in cytoskeleton maintenance [88]. Previous studies revealed that ACTG1 has a regulating effect in the myogenic cell migration, which is necessary for skeletal muscle formation [1,89]. Other studies found that knocking out ACTG1 leads to growth delay and skeletal myopathy in mice [90,91].…”

Section: Discussionmentioning

confidence: 99%

Integrated Transcriptome Analysis of miRNAs and mRNAs in the Skeletal Muscle of Wuranke Sheep

Yun,

Wu,

et al. 2023

Genes

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MicroRNAs (miRNAs) are regarded as important regulators in skeletal muscle development. To reveal the regulatory roles of miRNAs and their target mRNAs underlying the skeletal muscle development of Wuranke sheep, we investigated the miRNA and mRNA expression profiles in the biceps femoris of these sheep at the fetal (3 months of gestation) and 3- and 15-month-old postnatal stages. Consequently, a total of 1195 miRNAs and 24,959 genes were identified. Furthermore, 474, 461, and 54 differentially expressed miRNAs (DEMs) and 6783, 7407, and 78 differentially expressed genes (DEGs) were detected among three comparative groups. Functional analysis demonstrated that the target mRNAs of the DEMs were enriched in multiple pathways related to muscle development. Moreover, the interactions among several predicted miRNA–mRNA pairs (oar-miR-133-HDAC1, oar-miR-1185-5p-MYH1/HADHA/OXCT1, and PC-5p-3703_578-INSR/ACTG1) that potentially affect skeletal muscle development were verified using dual-luciferase reporter assays. In this study, we identified the miRNA and mRNA differences in the skeletal muscle of Wuranke sheep at different developmental stages and revealed that a series of candidate miRNA–mRNA pairs may act as modulators of muscle development. These results will contribute to future studies on the function of miRNAs and their target mRNAs during skeletal muscle development in Wuranke sheep.

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Section: Discussionmentioning

confidence: 99%

Integrated Transcriptome Analysis of miRNAs and mRNAs in the Skeletal Muscle of Wuranke Sheep

Yun,

Wu,

et al. 2023

Genes

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“…Domenighetti et al demonstrated that FHL1-null mice develop an age-dependent myopathy associated with myofibrillar and intermyofibrillar disorganization (Domenighetti et al 2014 ). FHL1 was recently found as part of a complex which binds gamma-actin and NMHC IIB in vivo and in vitro (Wang et al 2013 ). Both NRAP and FHL1 are partners of NMHC IIB (Lu and Horowits 2008 ; Wang et al 2013 ) and the patient’s variants in these genes could affect their interactions with NMHC IIB, with possible implications in myofibrillar assembly.…”

Section: Discussionmentioning

confidence: 99%

Exome sequencing identifies variants in two genes encoding the LIM-proteins NRAP and FHL1 in an Italian patient with BAG3 myofibrillar myopathy

D’Avila

Meregalli

Lupoli

et al. 2016

J Muscle Res Cell Motil

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Myofibrillar myopathies (MFMs) are genetically heterogeneous dystrophies characterized by the disintegration of Z-disks and myofibrils and are associated with mutations in genes encoding Z-disk or Z-disk-related proteins. The c.626 C > T (p.P209L) mutation in the BAG3 gene has been described as causative of a subtype of MFM. We report a sporadic case of a 26-year-old Italian woman, affected by MFM with axonal neuropathy, cardiomyopathy, rigid spine, who carries the c.626 C > T mutation in the BAG3 gene. The patient and her non-consanguineous healthy parents and brother were studied with whole exome sequencing (WES) to further investigate the genetic basis of this complex phenotype. 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. Quantitative real time PCR, immunohistochemistry and Western blot analysis of the patient’s muscular biopsy showed the absence of NRAP expression and FHL1 accumulation in aggregates in the affected skeletal muscle tissue. Molecular dynamic analysis of the mutated FHL1 domain showed a modification in its surface charge, which could affect its capability to bind its target proteins. To our knowledge this is the first study reporting, in a BAG3 MFM, the simultaneous presence of genetic variants in the BAG3 and FHL1 genes (previously described as independently associated with MFMs) and linking the NRAP gene to MFM for the first time.

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“…FHL1 mutations have been identified in a spectrum of human skeletal and cardiac muscle diseases 21 - 24 . FHL1 could alter cytoskeleton and cell shape by binding with PDZ and LIM domain protein 1 (PDLIM1), Gelsolin (GSN), gamma-actin (ACTG) and a-actin (ACTN1) 37 , 38 . In rat aortic smooth muscle cells (SMCs) FHL1 knockdown could significantly inhibit the proliferation of SMCs but exerted no significant effect on cell apoptosis 25 .…”

Section: Discussionmentioning

confidence: 99%

Up-regulated FHL1 Expression Maybe Involved in the Prognosis of Hirschsprung's Disease

Wang¹,

Gu²,

Yang³

et al. 2014

Int. J. Med. Sci.

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Background: In a subset of patients with Hirschsprung's disease (HSCR), gastrointestinal motor dysfunction persisted long after surgical correction. Gastrointestinal motility is achieved through the coordinated activity of the enteric nervous system, interstitial cells of Cajal, and smooth muscle (SMC) cells. Inhibition of four-and-a-half LIM protein-1 (Fhl1) expression by siRNA significantly decreases pulmonary artery SMCs migration and proliferation. Furthermore when up-expressing FHL1 in atrial myocytes, K (+) current density markedly increases, therefore changing myocytes' response to an electrical stimulus. However whether FHL1 in colon SMCs (the final effector organ) influences intestinal motility in HSCR patients has not been clarified. Methods: FHL1 mRNA and protein expressions were analyzed in 32 HSCR colons and 4 normal colons. Results: Smooth muscle layers were thicken and disorganized in HSCR. FHL1 was expressed in the ganglion cells of the myenteric plexus, submucosa, as well as in the longitudinal and circular muscle layer of the ganglionic colon. FHL1 mRNA relative expression level in aganglionic colons was 1.06±0.49 (ganglionic colon relative expression level was 1) (P=0.44). FHL1 protein gray level relative to GAPDH in normal colons was 0.83±0.09. FHL1 expression level in ganglionic colon (1.66±0.30) or aganglionic colon (1.81±0.35) was significantly higher than that in normal colons (P=0.045 and P=0.041, respectively). Meanwhile, we found FHL1 expression in aganglionic colon was slightly stronger than that in ganglionic colon (P=0.036). Conclusion: These data suggested that up-regulated FHL1 in smooth muscle in HSCR might be associated with intestinal wall remodeling in HSCR and might be one of the risk factors for gastrointestinal motor dysfunction.

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Identification of an FHL1 Protein Complex Containing Gamma-Actin and Non-Muscle Myosin IIB by Analysis of Protein-Protein Interactions

Cited by 4 publications

References 50 publications

Integrated Transcriptome Analysis of miRNAs and mRNAs in the Skeletal Muscle of Wuranke Sheep

Integrated Transcriptome Analysis of miRNAs and mRNAs in the Skeletal Muscle of Wuranke Sheep

Exome sequencing identifies variants in two genes encoding the LIM-proteins NRAP and FHL1 in an Italian patient with BAG3 myofibrillar myopathy

Up-regulated FHL1 Expression Maybe Involved in the Prognosis of Hirschsprung's Disease

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