NEB Antibody

Code CSB-PA324989LA01HU
Size US$166
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Image
  • IHC image of CSB-PA324989LA01HU diluted at 1:600 and staining in paraffin-embedded human lung cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

  • IHC image of CSB-PA324989LA01HU diluted at 1:600 and staining in paraffin-embedded human skeletal muscle tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

  • Immunofluorescence staining of HepG2 cells with CSB-PA324989LA01HU at 1:200, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) NEB Polyclonal antibody
Uniprot No.
Target Names
NEB
Alternative Names
DKFZp686C1456 antibody; FLJ11505 antibody; FLJ36536 antibody; FLJ39568 antibody; FLJ39584 antibody; NEB 177D antibody; NEB antibody; NEB177D antibody; NEBU_HUMAN antibody; Nebulin antibody; NEM 2 antibody; NEM2 antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Nebulin protein (245-373AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The NEB Antibody (Product code: CSB-PA324989LA01HU) is Non-conjugated. For NEB Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA324989LB01HU NEB Antibody, HRP conjugated ELISA
FITC CSB-PA324989LC01HU NEB Antibody, FITC conjugated
Biotin CSB-PA324989LD01HU NEB Antibody, Biotin conjugated ELISA
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
Tested Applications
ELISA, IHC, IF
Recommended Dilution
Application Recommended Dilution
IHC 1:500-1:1000
IF 1:200-1:500
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.

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Target Background

Function
This giant muscle protein may be involved in maintaining the structural integrity of sarcomeres and the membrane system associated with the myofibrils. Binds and stabilize F-actin.
Gene References into Functions
  1. NEB gene should be included in genetic panels for fetal akinesia/arthrogryposis multiplex congenital cases. PMID: 27933661
  2. We examined a Chinese strabismus pedigree with the parents unaffected and 2 offspring affected. Whole-exome sequencing and bioinformatics filtering identified 2 variants including Abelson helper integration site 1 (AHI1) gene and nebulin (NEB) gene. c.A914G mutation was found in nebulin (NEB) gene. PMID: 28391287
  3. Shorter than normal thin filament length contributes to the impaired force generation in patients with thin filament myopathy, but only in those who harbor specific mutations in NEB or ACTA1. PMID: 27074222
  4. New NEB mutations were found in 8 of 10 patients with nemaline myopathy. PMID: 27105866
  5. recurrent NEB TRI copy number variation was found in 13% of the families with nemaline myopathy and in 10% of the controls. PMID: 26197980
  6. Prominent foot-drop was associated with NEB gene mutations in three patients with core-rod myopathy. PMID: 26403434
  7. Identified two novel, compound-heterozygous variants in the nebulin gene after a 30 year clinical history, which cause a classical childhood type of nemaline myopathy. PMID: 25740301
  8. Since the patients are characterized by generalized muscle weakness together with neurodevelopmental phenotypes, it is suggested that NEB mutations could manifest more diverse phenotypes than those previously described. PMID: 25296583
  9. indicates that nebulin tolerates substantial changes in its amino acid sequence, providing an explanation as to why variants in such a large gene result in relatively rare disorders PMID: 25205138
  10. Mutations in NEB gene is associated with stress fracture. PMID: 25023003
  11. This study demonistrated that Muscle histopathology in nebulin-related nemaline myopathy: ultrastrastructural findings correlated to disease severity. PMID: 24725366
  12. We identify the SH3 domains of nebulin and nebulette as novel ligands of proline-rich regions of Xin and XIRP2 PMID: 23985323
  13. Data suggest that for some filament-forming desmin mutants, the molecular etiology of desminopathy results from subtle deficiencies in their association with nebulin, a major actin-binding filament protein of striated muscle. PMID: 23615443
  14. A nebulin-based nemaline myopathy model is characterized in transgenic mice following deletion of exon 55 in nebulin. PMID: 23715096
  15. Multiple isoforms of nebulin expressed in skeletal muscle and brain may have a role as actin filament stabilizer or length regulator in neurons of the human brain PMID: 22941678
  16. Ultrasonographic findings suggestive of a myopathy and a carrier state for the NEB exon 55 deletion in one of the parents should trigger a thorough investigation for nemaline. PMID: 22367672
  17. distal nemaline myopathy caused by four different compound heterozygous nebulin mutations PMID: 21724397
  18. The mechanics as well as the X-ray diffraction patterns of human membrane-permeabilized single muscle fibers expressing nebulin mutations are reported. PMID: 21350120
  19. Data revealed markedly reduced nebulin protein levels in muscle from nemaline myopathy patients, whereas levels of other thin filament-based proteins were not significantly altered. PMID: 19944167
  20. Nebulin regulates thin filament architecture by a mechanism that includes stabilizing the filaments and preventing actin depolymerization. PMID: 20498015
  21. SH3 domain of nebulin binds selectively to type II peptides: theoretical prediction and experimental validation PMID: 11851340
  22. Lack of the C-terminal domain of nebulin in a patient with nemaline myopathy. PMID: 11994971
  23. nebulin colocalizes with desmin in a Z-line-associated, striated pattern, thereby forming a lateral linkage system which contributes to maintain adjacent Z-lines in register as shown by immunofluorescence studies PMID: 12064939
  24. Mutations caused absence of the C-terminal part of nebulin resulting in severe congenital form of nemaline myopathy. PMID: 12207937
  25. Nebulin is targeted and oriented through titin and myopalladin signaling during sarcomere assembly PMID: 12482578
  26. 45 novel mutations within the nubulin gene are associated with nemaline myopathy. PMID: 16917880
  27. These data suggest that N-terminal superrepeat nebulin modules are incapable of supporting interactions with the cardiac myofilaments; whereas the C-terminal nebulin modules can. PMID: 17275809
  28. These data suggest a model in which archvillin attaches directly to the Z-line of skeletal muscle through an interaction with the nebulin C-terminus. PMID: 18639526
  29. In all but two of eight homozygous patients with nebulin mutations, the clinical picture was more severe than in typical nemaline myopathy. PMID: 19232495
  30. Nebulin is thought to serve as both a length-regulating protein ruler and calcium/calmodulin-mediated regulatory protein on the thin filaments of the skeletal muscle sarcomere. PMID: 11425319

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Involvement in disease
Nemaline myopathy 2 (NEM2)
Subcellular Location
Cytoplasm, myofibril, sarcomere. Cytoplasm, cytoskeleton.
Tissue Specificity
Muscle specific. Located in the thin filament of striated muscle.
Database Links

HGNC: 7720

OMIM: 161650

KEGG: hsa:4703

UniGene: Hs.588655

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