NEBL Antibody, FITC conjugated

Code CSB-PA015673LC01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) NEBL Polyclonal antibody
Uniprot No.
Target Names
NEBL
Alternative Names
NEBL antibody; LNEBLNebulette antibody; Actin-binding Z-disk protein antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Nebulette protein (438-533AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
FITC
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
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.
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Function
Binds to actin and plays an important role in the assembly of the Z-disk. May functionally link sarcomeric actin to the desmin intermediate filaments in the heart muscle sarcomeres. Isoform 2 might play a role in the assembly of focal adhesion.
Gene References into Functions
  1. Survival analysis suggested that overexpressed NEBL in patients with colorectal cancer was associated with a positive prognosis for overall survival. PMID: 29257257
  2. LASP2 may play a significant role in suppressing CRC progression and provided a novel biomarker for CRC therapy PMID: 28606091
  3. The levels of phosphorylated FAK (Tyr397 and Tyr925) were increased after overexpressing Lasp2 and were downregulated by transfecting Lasp2-siRNA. PMID: 28667800
  4. predisposition to multibacillary leprosy in Vietnam is associated with CUBN and NEBL common variants in the chromosome 10p13 linkage region PMID: 24563210
  5. We identify the SH3 domains of nebulin and nebulette as novel ligands of proline-rich regions of Xin and XIRP2 PMID: 23985323
  6. Data indicate that lasp-2 interacts with the focal adhesion proteins vinculin and paxillin. PMID: 23389630
  7. Report oncogenic potential of MLL-NEBL and NEBL-MLL fusion genes in acute myeloid leukemia. PMID: 23340173
  8. Different mutations in nebulette transgene trigger a pathological cascade leading to endocardial fibroelastosis and dilated cardiomyopathy in mutant embryonic mouse hearts. PMID: 20951326
  9. our data demonstrate the importance of this cardiac-specific nebulin isoform in myofibril organization and function. Our data demonstrate that nebulette plays a significant role in the structure and stability of the cardiac Z-line. PMID: 11822876
  10. LIM-nebulette, Lasp-1, and zyxin may play an important role in the organization of focal adhesions PMID: 15004028
  11. filamin-C, a known component of striated muscle Z-lines, interacts with nebulette modules PMID: 17987659
  12. the importance of the nebulette-TPM interactions in the maintenance and stability of the thin filaments. PMID: 18823973

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Subcellular Location
[Isoform 2]: Cytoplasm.
Tissue Specificity
Abundantly expressed in cardiac muscle, but not in skeletal or smooth muscle. Localized to Z-lines in cardiac cells and to dense bodies in nonmuscle cells. Isoform 2 is expressed in non-muscle cells such as in fibroblasts.
Database Links

HGNC: 16932

OMIM: 605491

KEGG: hsa:10529

STRING: 9606.ENSP00000366326

UniGene: Hs.5025

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