LDB3 Antibody
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中文名稱：LDB3兔多克隆抗體
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貨號：CSB-PA012831ESR1HU
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規格：￥440
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圖片：
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Western blot
All lanes: LDB3 antibody at 3.21μg/ml
Lane 1 : Hela whole cell lysate
Lane 2 : Raji whole cell lysate
Lane 3 : MCF-7 whole cell lysate
Lane 4 : A431 whole cell lysate
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 78, 67, 51, 43, 36, 31, 79 kDa
Observed band size: 67, 51 kDa -
Immunohistochemistry of paraffin-embedded human skeletal muscle tissue using CSB-PA012831ESR1HU at dilution of 1:100
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) LDB3 Polyclonal antibody
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Uniprot No.：
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基因名：LDB3
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別名：CMD1C antibody; CYPHER antibody; HGNC:15710 antibody; KIAA01613 antibody; KIAA0613 antibody; Ldb3 antibody; LDB3_HUMAN antibody; LDB3Z1 antibody; LDB3Z4 antibody; LIM domain binding 3 antibody; LIM domain binding protein 3 antibody; LIM domain-binding protein 3 antibody; LVNC3 antibody; ORACLE antibody; PDLIM6 antibody; PDZ and LIM domain 6 antibody; Protein cypher antibody; Z band alternatively spliced PDZ motif antibody; Z band alternatively spliced PDZ motif protein antibody; Z-band alternatively spliced PDZ-motif protein antibody; ZASP antibody
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宿主：Rabbit
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反應種屬：Human
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免疫原：Recombinant Human LIM domain-binding protein 3 protein (1-283AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：Antigen Affinity Purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
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產品提供形式：Liquid
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應用范圍：ELISA, WB, IHC
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推薦稀釋比：
Application Recommended Dilution WB 1:1000-1:5000 IHC 1:20-1:200 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
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靶點詳情
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功能：May function as an adapter in striated muscle to couple protein kinase C-mediated signaling via its LIM domains to the cytoskeleton.
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基因功能參考文獻：
- Interaction is demonstrated between ZASP and two new binding partners both of which have roles in signalling, regulation of gene expression and muscle differentiation; the mechanosensing protein Ankrd2 and the tumour suppressor protein p53. PMID: 24647531
- p.(D117N) variant in Cypher/ZASP is not a causative mutation for dilated cardiomyopathy and ventricular arrhythmias. PMID: 26419279
- A novel heterozygous missense mutation (p.N155H) in a highly conserved PDZ-like motif of ZASP was identified. The results indicate that typical ZASP-MFM presenting with late-onset distal myopathy is commonly associated with mutations in PDZ-like motif of ZASP. PMID: 27546599
- The molecular basis for high-affinity binding of ZASP to G-Actin has been described. PMID: 28349680
- this is the first family with Arrhythmogenic right ventricular cardiomyopathy where a mutation in LDB3 is associated with Arrhythmogenic right ventricular cardiomyopathy PMID: 25041374
- Study identified abnormal inclusion of LDB3 exon 11 specific to myotonic dystrophy type at the RNA and protein level; inclusion changed the affinity of LDB3 for PKC, indicating that exon 11 may contribute to the activation of PKC in DM1 PMID: 24878509
- results show that MFM-associated ZASP mutations in the actin-binding domain have deleterious effects on the core structure of the Z-discs in skeletal muscle. PMID: 24668811
- Z-band alternatively spliced PDZ motif protein (ZASP) is the major O-linked beta-N-acetylglucosamine-substituted protein in human heart myofibrils PMID: 23271734
- This results of this indicted that One patient harbored the missense mutation c.1719G>A (p.V566M) in the ZASP gene. PMID: 22349865
- ZASP1 can form protein complex with telethonin/T-Cap and Na(v)1.5. The left ventricular noncompaction-specific ZASP1 mutation can cause loss of function of Na(v)1.5, without significant alteration of the cytoskeletal protein complex. PMID: 22929165
- TNNT3 and LDB3 showed abnormal splicing and appeared more pronounced in myotonic dystrophies type 2 relative to myotonic dystrophies type 1. PMID: 20066428
- the D626N mutation of Cypher/ZASP increases the affinity of the LIM domain for protein kinase C, which may be related to pathogenesis of dilated cardiomyopathy PMID: 14660611
- ZASP/Cypher internal fragments containing either ZM exon 4 or 6 co-localized with alpha-actinin in cultured myoblasts and nonmuscle cells. PMID: 16476425
- Tafazzins are essential during fetal and early post-natal life. PMID: 17394203
- ZASP/Cypher anchors PGM1 to Z-disc under conditions of stress. The impaired binding of PGM1 to ZASP/Cypher might be involved in the pathogenesis of dilated cardiomyopathy. PMID: 19377068
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相關疾?。?/div>Cardiomyopathy, dilated 1C, with or without left ventricular non-compaction (CMD1C); Left ventricular non-compaction 3 (LVNC3); Myopathy, myofibrillar, 4 (MFM4)亞細胞定位：Cytoplasm, perinuclear region. Cell projection, pseudopodium. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line. Note=Localized to the cytoplasm around nuclei and pseudopodia of undifferentiated cells and detected throughout the myotubes of differentiated cells. Colocalizes with ACTN2 at the Z-lines.組織特異性：Expressed primarily in skeletal muscle and to a lesser extent in heart. Also detected in brain and placenta.數據庫鏈接：
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