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Cleaved-LMNA (D230) Antibody

  • 中文名稱:
    Cleaved-LMNA (D230)兔多克隆抗體
  • 貨號:
    CSB-PA000047
  • 規(guī)格:
    ¥1090
  • 圖片:
    • Western Blot analysis of HeLa cells using Cleaved-Lamin A (D230) Polyclonal Antibody
  • 其他:

產(chǎn)品詳情

  • Uniprot No.:
  • 基因名:
  • 別名:
    70 kDa lamin antibody; Cardiomyopathy dilated 1A (autosomal dominant) antibody; CDCD1 antibody; CDDC antibody; CMD1A antibody; CMT2B1 antibody; EMD2 antibody; FPL antibody; FPLD antibody; FPLD2 antibody; HGPS antibody; IDC antibody; Lamin A antibody; Lamin A/C antibody; Lamin A/C like 1 antibody; Lamin antibody; Lamin C antibody; lamin-a antibody; Lamin-A/C antibody; LDP1 antibody; LFP antibody; LGMD1B antibody; Limb girdle muscular dystrophy 1B (autosomal dominant) antibody; LMN 1 antibody; LMN A antibody; LMN C antibody; LMN1 antibody; LMNA antibody; LMNA_HUMAN antibody; LMNC antibody; LMNL1 antibody; Prelamin A/C antibody; PRO1 antibody; Renal carcinoma antigen NY REN 32 antibody; Renal carcinoma antigen NY-REN-32 antibody; Renal carcinoma antigen NYREN32 antibody
  • 宿主:
    Rabbit
  • 反應(yīng)種屬:
    Human,Mouse,Rat
  • 免疫原:
    Synthesized peptide derived from the Internal region of Human Lamin A.
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標(biāo)記方式:
    Non-conjugated
  • 抗體亞型:
    IgG
  • 純化方式:
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 產(chǎn)品提供形式:
    Liquid
  • 應(yīng)用范圍:
    WB, IHC, ELISA
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:500-1:2000
    IHC 1:100-1:300
    ELISA 1:10000
  • Protocols:
  • 儲存條件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期:
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評價

靶點詳情

  • 功能:
    Lamins are components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to provide a framework for the nuclear envelope and may also interact with chromatin. Lamin A and C are present in equal amounts in the lamina of mammals. Recruited by DNA repair proteins XRCC4 and IFFO1 to the DNA double-strand breaks (DSBs) to prevent chromosome translocation by immobilizing broken DNA ends. Plays an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics. Required for normal development of peripheral nervous system and skeletal muscle and for muscle satellite cell proliferation. Required for osteoblastogenesis and bone formation. Also prevents fat infiltration of muscle and bone marrow, helping to maintain the volume and strength of skeletal muscle and bone. Required for cardiac homeostasis.; Prelamin-A/C can accelerate smooth muscle cell senescence. It acts to disrupt mitosis and induce DNA damage in vascular smooth muscle cells (VSMCs), leading to mitotic failure, genomic instability, and premature senescence.
  • 基因功能參考文獻(xiàn):
    1. Lamin A-C interaction with Nestin and its role in the tumor senescence.Nestin stabilizes lamin A-C to protect tumor cells from senescence. PMID: 30190500
    2. Among the 120 dilated cardiomyopathy patients 13 (10.8%) had LMNA variants. A novel recurrent LMNA E115M variant was the most frequent in familial DCM. PMID: 29386531
    3. lamin A/C interacts with Notch signaling, thereby influencing cellular differentiation, and point mutation in LMNA could halt this interaction PMID: 29040816
    4. Mutations in LMNA cause autosomal dominant severe heart disease, accounting for 10% of Dilated Cardiomyopathy . PMID: 29175975
    5. ZMPSTE24-dependent cleavage of prelamin A and the eight known disease-associated ZMPSTE24 missense mutations, were examined. PMID: 29794150
    6. The LMNA-NTRK1 fusion was likely the molecular driver of tumorigenesis and metastasis in this patient, and the observed effectiveness of crizotinib treatment provides clinical validation of this molecular target. PMID: 30134855
    7. Three heterozygous missense mutations were identified in unrelated patients - p. W520R (c.1558T > C), p.T528R (small es, Cyrillic.1583capital ES, Cyrillic > G) and p.R190P (c.569G > C). We consider these variants as pathogenic, leading to isolated DCM with conduction defects or syndromic DCM forms with limb-girdle muscular dystrophy and Emery- Dreifuss muscular dystrophy. PMID: 29770364
    8. The functional integrity of lamin and nesprin-1 is thus required to modulate the FHOD1 activity and the inside-out mechanical coupling that tunes the cell internal stiffness to match that of its soft, physiological-like environment. PMID: 28455503
    9. The role of 1B and 2B domains in modulating elastic properties of lamin A. PMID: 27301336
    10. progerin is upregulated in human dilated cardiomyopathy hearts and strongly correlates with left ventricular remodeling PMID: 29702688
    11. Data indicate that patients with truncation mutations in LMNA (lamin A/C) had an earlier occurrence of cardiac conduction disturbance and low left ventricular ejection fraction, than those with missense mutations. PMID: 29237675
    12. A novel truncating LMNA mutation associated with Cardiac conduction disorders and dilated cardiomyopathy was discovered in this family characterized by gender differences in clinical severity in LMNA carriers PMID: 29628476
    13. We find no evidence for an elevated mutation rate in progerin-expressing cells. We conclude that the cellular defect in HGPS cells does not lie in the repair of DNA damage per se. PMID: 28477268
    14. pathogenic gene mutations in LMNA and MYBPC3 alter RNA splicing and may have a role in heart disease PMID: 28679633
    15. Patients with the heterozygous LMNA p.T10I mutation have distinct clinical features and significantly worse metabolic complications compared with other patients with atypical progeroid syndrome as well as patients with Hutchinson-Gilford progeria syndrome. PMID: 29267953
    16. Results suggest that lamin A/C might constitute a type of epithelial marker that better signifies EMT and MET in prostate cancer tissue, since a decrease in lamin A/C expression in Gleason score (GS) 4 is likely associated with the EMT process, while the re-expression of lamin A/C in GS 5 is likely linked with MET. PMID: 29665450
    17. Using cardiomyocytes derived from human induced pluripotent stem cells carrying different LMNA mutations as model for dilated cardiomyopathy, demonstrate that PTC124 induces translational read-through over the premature stop codon and restores production of the full-length protein. PMID: 28754655
    18. This study represents a comprehensive report on relative frequency of CMD in the UK population, indicating MDC1A as the most common CMD subtype (37.35%). PMID: 28688748
    19. in differentiating myoblasts, nuclear HSPB2 compartments sequester lamin A. PMID: 28854361
    20. A mutation in the gene encoding Lamin A/C (LMNAp.R331Q ) led to reduced maximal force development through secondary disease remodelling in patients suffering from dilated cardiomyopathy. PMID: 28436080
    21. In embryonic cells upregulation of lamin A disturbs lamin C, which may influence gene expression. PMID: 27534416
    22. our data demonstrate the occurrence of lamin A/NF-Y interaction and suggest a possible role of this protein complex in regulation of NF-Y function in cell proliferation. PMID: 27793050
    23. Findings provide evidence that lamin A mutants (called progerin) activates the DNA damage response pathway and that dysregulation of this pathway may be responsible for the development of cardiovascular pathology in patients with Hutchinson-Gilford progeria syndrome. PMID: 28423660
    24. Type-2 familial partial lipodystrophy (FPLD2) is a rare autosomal dominant lipodystrophic disorder due to mutations in LMNA. PMID: 28408391
    25. The metabolic features of women with the Dunnigan variety of familial partial lipodystrophy, caused by several missense mutations of LMNA, are reported. PMID: 28443701
    26. UVA-induced progerinlamin A complex formation was largely responsible for suppressing 53BP1-mediated NHEJ DSB repair activity. The present study is the first to demonstrate that UVA-induced progerin upregulation adversely affects 53BP1-mediated NHEJ DSB repair in human keratinocytes via progerinlamin A complex formation. PMID: 28498430
    27. Suggest NF-YAs and lamin A expression levels as novel potential biomarkers useful to identify G1 endometrial carcinoma patients with risk of recurrence. PMID: 27974701
    28. Finally, we demonstrate Lamins as the major factors in reliable miR-218 and miR-129 functions for breast cancer progression. Our findings uncover a new miRNA-mediated regulatory network for different Lamins and provide a potential therapeutic target for breast cancer. PMID: 29378184
    29. Data indicates that D243Gfs*4 LMNA as a mutation causing a severe form of cardiomyopathy with conduction defects, and suggest CX43 downregulation as a possible molecular mechanism leading to the conduction defects observed in mutation carriers. PMID: 29197877
    30. two novel RNA isoforms of LMNA produced through alternative splicing PMID: 28857661
    31. Lamin A/C is an autoantigen in Han Chinese patients with confirmed Sjogren's syndrome. Lamin A/C shares similar epitopes with U1RNP. PMID: 27835913
    32. it was demonstrated that suspension state promoted the reattachment of breast tumor cells by up-regulating lamin A/C via cytoskeleton disruption. These findings highlight the important role of suspension state for tumor cells in tumor metastasis. PMID: 28919351
    33. In this report we show that increased self-association propensity of mutant LA modulates the LA-LB1 interaction and precludes the formation of an otherwise uniform laminar network. Our results might highlight the role of homotypic and heterotypic interactions of LA in the pathogenesis of DCM and hence laminopathies in the broader sense. PMID: 28844980
    34. Familial partial lipodystrophy type 2 (FPLD2) is caused by an autosomal dominant mutation in the LMNA gene. FPLD2-adipocytes appear to accumulate markers of autophagy and catabolize triglycerides at higher levels than control adipocytes. PMID: 29108996
    35. we demonstrate that BAF is necessary to modulate prelamin A effects on chromatin structure PMID: 26701887
    36. Dysmorphic nuclei in patients with an LMNA mutation correlate with the age of heart disease presentation. PMID: 29149195
    37. These results suggest that the nuclear lamins and progerin have marginal roles in the activation of the antioxidant Nrf2 response to arsenic and cadmium. PMID: 28229933
    38. developed a proteomic analysis of plasma samples from a family showing history of dilated cardiomyopathy caused by a LMNA mutation, which may lead to premature death or cardiac transplant PMID: 27457270
    39. Exome sequencing of the proband revealed an extremely rare missense heterozygous variant c.1711_1712CG>TC; p.(Arg571Ser) in LMNA which was confirmed by Sanger sequencing in both the patients. Interestingly, the mutation had no effect on mRNA splicing or relative expression of lamin A or C mRNA and protein in the lymphoblasts PMID: 28686329
    40. Case Report: pathogenic LMNA mutation gives unifying diagnosis explaining arrhythmogenic right ventricular cardiomyopathy and Charcot-Marie-Tooth type 2B1 phenotypes. PMID: 27405450
    41. Standard Sanger sequencing of LMNA exon 11 DNA from blood-derived WBCs and cultured skin fibroblasts sequenced at passages 1, 3 and 8 detected differing progerin-producing mutations in the same nucleotide of the exon 11 intronic splice donor site (see online supplementary figure. PMID: 27920058
    42. the CNOT1-LMNA-Hedgehog signaling pathway axis exerts an oncogenic role in osteosarcoma progression, which could be a potential target for gene therapy. PMID: 28188704
    43. Pathogenic variants in the LMNA gene responsible for nearly 10%-15%% of Familial Dilated Cardiomyopathy cases. PMID: 27736720
    44. low lamin A but not lamin C expression in pleural metastatic cells could represent a major actor in the development of metastasis, associated with epithelial to mesenchymal transition and could account for a pejorative factor correlated with a poor Performance status. PMID: 28806747
    45. These results propose a mechanism for progerin-induced genome instability and accelerated replicative senescence in Hutchinson-Gilford progeria syndrome. PMID: 28515154
    46. LmnA binds AIMP3 via its extreme C-terminus. Together these findings provide a structural insight for understanding the interaction between AIMP3 and LmnA in AIMP3 degradation. PMID: 28797100
    47. The R482W mutation results in a loss of function of differentiation-dependent lamin A binding to the MIR335 locus and epigenetic regulation of adipogenesis. PMID: 28751304
    48. Pathogenic variants of the LMNA gene were determined in nine families with familial partial lipodystrophy PMID: 28641778
    49. The interaction of progerin with lamin A/C contributes to the development of the senescence phenotype of Hutchinson-Gilford progeria syndrome and aged cells. PMID: 27617860
    50. we expressed a LEMD2 transgene alone or in combination with lamin C in these cells and observed no restoration of peripheral heterochromatin in either case. We conclude that in contrary to the B-tether, the A-tether has a more intricate composition and consists of multiple components that presumably vary, at differing degrees of redundancy, between cell types and differentiation stages PMID: 28056360

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  • 相關(guān)疾病:
    Emery-Dreifuss muscular dystrophy 2, autosomal dominant (EDMD2); Emery-Dreifuss muscular dystrophy 3, autosomal recessive (EDMD3); Cardiomyopathy, dilated 1A (CMD1A); Lipodystrophy, familial partial, 2 (FPLD2); Limb-girdle muscular dystrophy 1B (LGMD1B); Charcot-Marie-Tooth disease 2B1 (CMT2B1); Hutchinson-Gilford progeria syndrome (HGPS); Cardiomyopathy, dilated, with hypergonadotropic hypogonadism (CMDHH); Mandibuloacral dysplasia with type A lipodystrophy (MADA); Lethal tight skin contracture syndrome (LTSCS); Heart-hand syndrome Slovenian type (HHS-Slovenian); Muscular dystrophy congenital LMNA-related (MDCL)
  • 亞細(xì)胞定位:
    Nucleus. Nucleus envelope. Nucleus lamina. Nucleus, nucleoplasm. Nucleus matrix. Note=Farnesylation of prelamin-A/C facilitates nuclear envelope targeting and subsequent cleavage by ZMPSTE24/FACE1 to remove the farnesyl group produces mature lamin-A/C, which can then be inserted into the nuclear lamina. EMD is required for proper localization of non-farnesylated prelamin-A/C.; [Isoform C]: Nucleus speckle.
  • 蛋白家族:
    Intermediate filament family
  • 組織特異性:
    In the arteries, prelamin-A/C accumulation is not observed in young healthy vessels but is prevalent in medial vascular smooth muscle cells (VSMCs) from aged individuals and in atherosclerotic lesions, where it often colocalizes with senescent and degener
  • 數(shù)據(jù)庫鏈接:

    HGNC: 6636

    OMIM: 115200

    KEGG: hsa:4000

    STRING: 9606.ENSP00000357283

    UniGene: Hs.594444



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