Gene expression profiling of fibroblasts in a family with LMNA-related cardiomyopathy reveals molecular pathways implicated in disease pathogenesis

Gene expression profiling of fibroblasts in a family with LMNA-related cardiomyopathy reveals molecular pathways implicated in disease pathogenesis

Background: Intermediate filament proteins that construct the nuclear lamina protein lamin cell including A / C is encoded by the gene LMNA, and is involved in fundamental processes such as nuclear structure, gene expression and signal transduction. LMNA mutations predominantly affect the mesoderm-derived cell lineages in diseases collectively referred to as laminopathies which include dilated cardiomyopathy with conduction defects, various forms of muscular dystrophy and premature aging syndromes as Hutchinson-Gilford Progeria Syndrome. At present, our understanding of the molecular mechanisms that regulate tissue-specific manifestations of laminopathies still limited.


Methods: To gain more insight into the molecular mechanisms of a novel splice-site mutations in LMNA (c.357-2A> G) in a family affected by heart disease, we do deep RNA sequencing and analysis for the nine lines derived fibroblast samples three patients with cardiomyopathy, three unaffected family members, and three unrelated individuals not affected. We validated our findings by the study of quantitative PCR and protein.


Results: We identified eight genes were significantly differentially expressed between fibroblasts mutant and non-mutant, which included downregulated factor insulin growth factor binding protein 5 (IGFBP5) in patient samples. ERK pathway analysis indicates involvement / MAPK signaling pathways consistent with previous studies. We found no significant differences in gene expression of lamin A / C and B-type lamins between groups. In the mutant fibroblasts, RNA-seq confirmed that only LMNA predominantly expressed wild-type allele, and Western blot showed normal levels of the protein lamin A / C


Conclusion:
IGFBP5 can contribute in maintaining homeostasis pathway signaling, which may cause the molecular and structural abnormalities is important in the network are not affected as fibroblasts. the mechanism of compensation of other nuclear membrane protein was not found. Our results also showed that only one copy of the wild allele kind enough to normal levels of the protein lamin A / C to maintain physiological functions in the cell type affected. This indicates that the affected cell types such as heart tissue may be more sensitive to haploinsufficiency of lamin A / C. These results provide insight into the molecular mechanisms of diseases with a possible explanation for tissue specificity LMNA related dilated cardiomyopathy.

 Gene expression profiling of fibroblasts in a family with LMNA-related cardiomyopathy reveals molecular pathways implicated in disease pathogenesis
Gene expression profiling of fibroblasts in a family with LMNA-related cardiomyopathy reveals molecular pathways implicated in disease pathogenesis

A novel multi-targeted tyrosine kinase inhibitor in combination with irinotecan anlotinib have in-vitro anti-tumor activity against lung cancer, small cell lung human

Anlotinib is a multi-targeted tyrosine kinase inhibitor developed independently in China. biological effects remain unclear in small cell lung cancer (SCLC). The current study aimed to evaluate the effects of anlotinib in combination with irinotecan in H446 and H2227 SCLC cell lines and provide new treatment strategies for SCLC. the growth of cells of the two cell lines was inhibited by anlotinib, irinotecan and the combination with a dose-dependent manner.

Human Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RD-FGF1-Hu-48Tests 48 Tests
EUR 311

Human Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RD-FGF1-Hu-96Tests 96 Tests
EUR 424

Mouse Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RD-FGF1-Mu-48Tests 48 Tests
EUR 429

Mouse Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RD-FGF1-Mu-96Tests 96 Tests
EUR 591

Porcine Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RD-FGF1-p-48Tests 48 Tests
EUR 494

Porcine Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RD-FGF1-p-96Tests 96 Tests
EUR 684

Rat Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RD-FGF1-Ra-48Tests 48 Tests
EUR 450

Rat Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RD-FGF1-Ra-96Tests 96 Tests
EUR 622

Bovine Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

DLR-FGF1-b-48T 48T
EUR 493
Description: A sandwich quantitative ELISA assay kit for detection of Bovine Fibroblast Growth Factor 1, Acidic (FGF1) in samples from serum, plasma or other biological fluids.

Bovine Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

DLR-FGF1-b-96T 96T
EUR 641
Description: A sandwich quantitative ELISA assay kit for detection of Bovine Fibroblast Growth Factor 1, Acidic (FGF1) in samples from serum, plasma or other biological fluids.

Chicken Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

DLR-FGF1-Ch-48T 48T
EUR 454
Description: A sandwich quantitative ELISA assay kit for detection of Chicken Fibroblast Growth Factor 1, Acidic (FGF1) in samples from serum, plasma or other biological fluids.

Chicken Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

DLR-FGF1-Ch-96T 96T
EUR 587
Description: A sandwich quantitative ELISA assay kit for detection of Chicken Fibroblast Growth Factor 1, Acidic (FGF1) in samples from serum, plasma or other biological fluids.

Human Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

DLR-FGF1-Hu-48T 48T
EUR 331
Description: A sandwich quantitative ELISA assay kit for detection of Human Fibroblast Growth Factor 1, Acidic (FGF1) in samples from serum, plasma, tissue homogenates, cell lysates, cell culture supernates or other biological fluids.

Human Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

DLR-FGF1-Hu-96T 96T
EUR 418
Description: A sandwich quantitative ELISA assay kit for detection of Human Fibroblast Growth Factor 1, Acidic (FGF1) in samples from serum, plasma, tissue homogenates, cell lysates, cell culture supernates or other biological fluids.

Mouse Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

DLR-FGF1-Mu-48T 48T
EUR 435
Description: A sandwich quantitative ELISA assay kit for detection of Mouse Fibroblast Growth Factor 1, Acidic (FGF1) in samples from serum, plasma, tissue homogenates, cell lysates, cell culture supernates or other biological fluids.

Mouse Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

DLR-FGF1-Mu-96T 96T
EUR 561
Description: A sandwich quantitative ELISA assay kit for detection of Mouse Fibroblast Growth Factor 1, Acidic (FGF1) in samples from serum, plasma, tissue homogenates, cell lysates, cell culture supernates or other biological fluids.

Porcine Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

DLR-FGF1-p-48T 48T
EUR 493
Description: A sandwich quantitative ELISA assay kit for detection of Porcine Fibroblast Growth Factor 1, Acidic (FGF1) in samples from serum, plasma, tissue homogenates, cell lysates, cell culture supernates or other biological fluids.

Porcine Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

DLR-FGF1-p-96T 96T
EUR 641
Description: A sandwich quantitative ELISA assay kit for detection of Porcine Fibroblast Growth Factor 1, Acidic (FGF1) in samples from serum, plasma, tissue homogenates, cell lysates, cell culture supernates or other biological fluids.

Rat Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

DLR-FGF1-Ra-48T 48T
EUR 454
Description: A sandwich quantitative ELISA assay kit for detection of Rat Fibroblast Growth Factor 1, Acidic (FGF1) in samples from serum, plasma, tissue homogenates, cell lysates, cell culture supernates or other biological fluids.

Rat Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

DLR-FGF1-Ra-96T 96T
EUR 587
Description: A sandwich quantitative ELISA assay kit for detection of Rat Fibroblast Growth Factor 1, Acidic (FGF1) in samples from serum, plasma, tissue homogenates, cell lysates, cell culture supernates or other biological fluids.

Bovine Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RDR-FGF1-b-48Tests 48 Tests
EUR 516

Bovine Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RDR-FGF1-b-96Tests 96 Tests
EUR 716

Chicken Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RDR-FGF1-Ch-48Tests 48 Tests
EUR 470

Chicken Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RDR-FGF1-Ch-96Tests 96 Tests
EUR 651

Human Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RDR-FGF1-Hu-48Tests 48 Tests
EUR 325

Human Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RDR-FGF1-Hu-96Tests 96 Tests
EUR 442

Mouse Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RDR-FGF1-Mu-48Tests 48 Tests
EUR 447

Mouse Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RDR-FGF1-Mu-96Tests 96 Tests
EUR 618

Porcine Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RDR-FGF1-p-48Tests 48 Tests
EUR 516

Porcine Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RDR-FGF1-p-96Tests 96 Tests
EUR 716

Rat Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RDR-FGF1-Ra-48Tests 48 Tests
EUR 470

Rat Fibroblast Growth Factor 1, Acidic (FGF1) ELISA Kit

RDR-FGF1-Ra-96Tests 96 Tests
EUR 651

FGF1

LF-PR013 10 ug
EUR 192
Description: FGF1 protein

FGF1 Antibody

1-CSB-PA008237
  • EUR 222.00
  • EUR 195.00
  • 100ug
  • 50ug
Description: A polyclonal antibody against FGF1. Recognizes FGF1 from Human, Mouse, Rat. This antibody is Unconjugated. Tested in the following application: IHC, ELISA;IHC:1/100-1/300.ELISA:1/10000

FGF1 Antibody

1-CSB-PA008615ESR1HU
  • EUR 222.00
  • EUR 335.00
  • 100ul
  • 50ul
Description: A polyclonal antibody against FGF1. Recognizes FGF1 from Human, Mouse. This antibody is Unconjugated. Tested in the following application: ELISA, WB, IHC, IF; Recommended dilution: WB:1:200-1:1000, IHC:1:20-1:500, IF:1:50-1:200

FGF1 Antibody

1-CSB-PA008615ESR2HU
  • EUR 222.00
  • EUR 335.00
  • 100ul
  • 50ul
Description: A polyclonal antibody against FGF1. Recognizes FGF1 from Human. This antibody is Unconjugated. Tested in the following application: ELISA, IHC, IF; Recommended dilution: IHC:1:20-1:500, IF:1:50-1:200

FGF1 Antibody

1-CSB-PA008615GA01HU
  • EUR 597.00
  • EUR 333.00
  • 150ul
  • 50ul
Description: A polyclonal antibody against FGF1. Recognizes FGF1 from Human, Mouse, Rat. This antibody is Unconjugated. Tested in the following application: ELISA, IHC

Fgf1 Antibody

1-CSB-PA008615HA01MO
  • EUR 317.00
  • EUR 335.00
  • 100ug
  • 50ug
Description: A polyclonal antibody against Fgf1. Recognizes Fgf1 from Human, Mouse. This antibody is Unconjugated. Tested in the following application: ELISA, WB, IHC; Recommended dilution: WB:1:500-1:2000, IHC:1:20-1:200

FGF1 Antibody

1-CSB-PA06379A0Rb
  • EUR 317.00
  • EUR 335.00
  • 100ug
  • 50ug
Description: A polyclonal antibody against FGF1. Recognizes FGF1 from Human. This antibody is Unconjugated. Tested in the following application: ELISA

FGF1 Antibody

1-CSB-PA954065
  • EUR 317.00
  • EUR 244.00
  • 100ul
  • 50ul
Description: A polyclonal antibody against FGF1. Recognizes FGF1 from Human, Mouse, Rat. This antibody is Unconjugated. Tested in the following application: ELISA, IHC;ELISA:1:2000-1:10000, IHC:1:50-1:200

FGF1 Antibody

1-CSB-PA449957
  • EUR 317.00
  • EUR 244.00
  • 100ul
  • 50ul
Description: A polyclonal antibody against FGF1. Recognizes FGF1 from Human, Mouse, Rat. This antibody is Unconjugated. Tested in the following application: ELISA, IHC;ELISA:1:1000-1:5000, IHC:1:25-1:100

FGF1 Antibody

36770-100ul 100ul
EUR 252

FGF1 siRNA

20-abx916815
  • EUR 551.00
  • EUR 732.00
  • 15 nmol
  • 30 nmol

FGF1 siRNA

20-abx916816
  • EUR 551.00
  • EUR 732.00
  • 15 nmol
  • 30 nmol

FGF1 siRNA

20-abx901949
  • EUR 551.00
  • EUR 732.00
  • 15 nmol
  • 30 nmol

FGF1 antibody

38145-100ul 100ul
EUR 252

FGF1 Antibody

ABD6124 100 ug
EUR 438

FGF1 Antibody

DF6124 200ul
EUR 304
Description: FGF1 Antibody detects endogenous levels of total FGF1.

pEGFP-FGF1

PVT17864 2 ug
EUR 258

anti-FGF1

YF-PA11765 50 ul
EUR 363
Description: Mouse polyclonal to FGF1

anti-FGF1

YF-PA11766 50 ug
EUR 363
Description: Mouse polyclonal to FGF1

anti-FGF1

YF-PA11767 100 ul
EUR 403
Description: Rabbit polyclonal to FGF1

anti-FGF1

YF-PA11768 100 ug
EUR 403
Description: Rabbit polyclonal to FGF1

anti-FGF1

YF-PA23703 50 ul
EUR 334
Description: Mouse polyclonal to FGF1

FGF1 antibody

10R-4098 100 ul
EUR 726
Description: Mouse monoclonal FGF1 antibody

FGF1 antibody

70R-17289 50 ul
EUR 435
Description: Rabbit polyclonal FGF1 antibody

FGF1 protein

30R-2095 10 ug
EUR 272
Description: Purified recombinant Human Acidic Fibroblast Growth Factor

FGF1 protein

30R-2096 25 ug
EUR 470
Description: Purified recombinant Human Acidic Fibroblast Growth Factor

FGF1 protein

30R-2102 1 mg
EUR 6005
Description: Purified recombinant Human Acidic Fibroblast Growth Factor

FGF1 protein

30R-2334 50 ug
EUR 325
Description: Purified recombinant Human FGF1 protein

FGF1 protein

30R-2335 50 ug
EUR 325
Description: Purified recombinant Mouse FGF1 protein

FGF1 antibody

70R-12251 100 ug
EUR 403
Description: Rabbit polyclonal FGF 1 antibody

FGF1 antibody

70R-13668 100 ug
EUR 365
Description: Affinity purified Rabbit polyclonal FGF1 antibody

FGF1 antibody

70R-13853 100 ug
EUR 322
Description: Affinity purified Rabbit polyclonal FGF1 antibody

Fgf1 antibody

70R-8011 50 ug
EUR 467
Description: Affinity purified rabbit polyclonal Fgf1 antibody

Fgf1 antibody

70R-8030 50 ug
EUR 467
Description: Affinity purified rabbit polyclonal Fgf1 antibody

FGF1 cloning plasmid

CSB-CL008615HU-10ug 10ug
EUR 233
Description: A cloning plasmid for the FGF1 gene.

Anti-FGF1 Antibody

RP1005 100ug/vial
EUR 334

Anti-FGF1 Antibody

PA1311 100ug/vial
EUR 294

Anti-FGF1 Antibody

PB9241 100ug/vial
EUR 294

Anti-FGF1 Antibody

PB9944 100ug/vial
EUR 334

Human FGF1 Antibody

32912-05111 150 ug
EUR 261

Anti-FGF1 antibody

STJ23650 100 µl
EUR 277
Description: The protein encoded by this gene is a member of the fibroblast growth factor (FGF) family. FGF family members possess broad mitogenic and cell survival activities, and are involved in a variety of biological processes, including embryonic development, cell growth, morphogenesis, tissue repair, tumor growth and invasion. This protein functions as a modifier of endothelial cell migration and proliferation, as well as an angiogenic factor. It acts as a mitogen for a variety of mesoderm- and neuroectoderm-derived cells in vitro, thus is thought to be involved in organogenesis. Multiple alternatively spliced variants encoding different isoforms have been described.

Anti-FGF1 antibody

STJ23651 100 µl
EUR 277
Description: The protein encoded by this gene is a member of the fibroblast growth factor (FGF) family. FGF family members possess broad mitogenic and cell survival activities, and are involved in a variety of biological processes, including embryonic development, cell growth, morphogenesis, tissue repair, tumor growth and invasion. This protein functions as a modifier of endothelial cell migration and proliferation, as well as an angiogenic factor. It acts as a mitogen for a variety of mesoderm- and neuroectoderm-derived cells in vitro, thus is thought to be involved in organogenesis. Multiple alternatively spliced variants encoding different isoforms have been described.

FGF1 Rabbit pAb

A0079-100ul 100 ul
EUR 308

FGF1 Rabbit pAb

A0079-200ul 200 ul
EUR 459

FGF1 Rabbit pAb

A0079-20ul 20 ul
EUR 183

FGF1 Rabbit pAb

A0079-50ul 50 ul
EUR 223

FGF1 Rabbit pAb

A0685-100ul 100 ul
EUR 308

FGF1 Rabbit pAb

A0685-200ul 200 ul
EUR 459

FGF1 Rabbit pAb

A0685-20ul 20 ul
EUR 183

FGF1 Rabbit pAb

A0685-50ul 50 ul
EUR 223

Human FGF1 Protein

abx060150-100ug 100 ug
EUR 509

FGF1 Polyclonal Antibody

A55415 100 µg
EUR 570.55
Description: Ask the seller for details

Fgf1 Polyclonal Antibody

A52780 100 µg
EUR 570.55
Description: reagents widely cited

anti- FGF1 antibody

FNab03087 100µg
EUR 548.75
Description: Antibody raised against FGF1

FGF1 Blocking Peptide

DF6124-BP 1mg
EUR 195

Anti-FGF1 antibody

PAab03087 100 ug
EUR 386

Anti-FGF1 (2E12)

YF-MA10321 100 ug
EUR 363
Description: Mouse monoclonal to FGF1

Anti-FGF1 (1F9)

YF-MA10322 100 ug
EUR 363
Description: Mouse monoclonal to FGF1

Anti-FGF1 (3F5)

YF-MA12986 100 ug
EUR 363
Description: Mouse monoclonal to FGF1

Fgf1 Blocking Peptide

33R-5638 100 ug
EUR 180
Description: A synthetic peptide for use as a blocking control in assays to test for specificity of Fgf1 antibody, catalog no. 70R-8011

Fgf1 Blocking Peptide

33R-1373 100 ug
EUR 180
Description: A synthetic peptide for use as a blocking control in assays to test for specificity of Irf2bp1 antibody, catalog no. 70R-9319

FGF1 Recombinant Protein

91-033 0.05 mg
EUR 364.25
Description: FGF acidic is a 17 kDa nonglycosylated member of the FGF family of mitogenic peptides. FGF acidic, which is produced by multiple cell types, stimulates the proliferation of all cells of mesodermal origin and many cells of neuroectodermal, ectodermal, and endodermal origin. It plays a number of roles in development, regeneration, and angiogenesis. FGF-acidic is a non-glycosylated heparin binding growth factor that is expressed in the brain, kidney, retina, smooth muscle cells, bone matrix, osteoblasts, astrocytes and endothelial cells. FGF-acidic has the ability to signal through all the FGF receptors.

FGF1 Recombinant Protein

91-039 0.05 mg
EUR 364.25
Description: FGF acidic, also known as ECGF, FGF-1and HBGF-1, is a non-glycosylated heparin binding growth factor that is expressed in the brain, kidney, retina, smooth muscle cells, bone matrix, osteoblasts, astrocytes and endothelial cells. It is a mitogenic peptide that is produced by multiple cell types and stimulates the proliferation of cells of mesodermal, ectodermal, and endodermal origin. Its association with heparan sulfate is a prerequisite for activation of FGF receptors. Internalized FGF acidic migrates to the nucleus where it is phosphorylated by nuclear PKC delta, exported to the cytosol, dephosphorylated, and degraded. Intracellular FGF acidic inhibits p53 activity and proapoptotic signaling.

FGF1 Recombinant Protein

92-269 0.05 mg
EUR 542.75
Description: FGF acidic, also known as ECGF, FGF-1and HBGF-1, is a non-glycosylated heparin binding growth factor that is expressed in the brain, kidney, retina, smooth muscle cells, bone matrix, osteoblasts, astrocytes and endothelial cells. It is a mitogenic peptide that is produced by multiple cell types and stimulates the proliferation of cells of mesodermal, ectodermal, and endodermal origin. Its association with heparan sulfate is a prerequisite for activation of FGF receptors. Internalized FGF acidic migrates to the nucleus where it is phosphorylated by nuclear PKC delta, exported to the cytosol, dephosphorylated, and degraded. Intracellular FGF acidic inhibits p53 activity and proapoptotic signaling.

After 72 hours of incubation, the degree of inhibition was greater in the combination group than all the single drug groups. Similar results were found when apoptosis was assessed after 12 h, but not after 6 hours of treatment. Compared with a single drug, drug combination suppressed the migration and invasion ability in two cell lines; However, there is no difference between anlotinib individual or irinotecan. Colony formation rates clearly lowered in the combination group.

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