Nebulin (NEB) ELISA Kits

NEB encodes nebulin, a giant protein component of the cytoskeletal matrix that coexists with the thick and thin filaments within the sarcomeres of skeletal muscle. Zusätzlich bieten wir Ihnen Nebulin Antikörper (28) und und viele weitere Produktgruppen zu diesem Protein an.

list all ELISA KIts Gen GeneID UniProt
NEB 4703 P20929
Anti-Maus NEB NEB 17996  
Anti-Ratte NEB NEB 311029  
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Katalog Nr. Reaktivität Sensitivität Bereich Bilder Menge Anbieter Lieferzeit Preis Details
Human 3.75 ng/mL 6.25 ng/mL - 400 ng/mL A typical standard curve 96 Tests Anmelden zum Anzeigen 13 bis 16 Tage

Weitere ELISA Kits für Nebulin Interaktionspartner

Zebrafish Nebulin (NEB) Interaktionspartner

  1. neb zebrafish mirror the genetic, clinical and pathological aspects of nemaline myopathy due to NEB mutation.

Human Nebulin (NEB) Interaktionspartner

  1. NEB gene should be included in genetic panels for fetal akinesia/arthrogryposis multiplex congenital cases.

  2. We examined a Chinese strabismus (zeige VANGL2 ELISA Kits) 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 (zeige AHI1 ELISA Kits)) gene and nebulin (NEB) gene. c.A914G mutation was found in nebulin (NEB) gene.

  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 (zeige ACTA1 ELISA Kits).

  4. New NEB mutations were found in 8 of 10 patients with nemaline myopathy.

  5. recurrent NEB TRI (zeige VANGL2 ELISA Kits) copy number variation was found in 13% of the families with nemaline myopathy and in 10% of the controls.

  6. Prominent foot-drop was associated with NEB gene mutations in three patients with core-rod myopathy.

  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.

  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.

  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

  10. Mutations in NEB gene is associated with stress fracture.

Rabbit Nebulin (NEB) Interaktionspartner

  1. Nebulin was solubilized by a salt solution containing 1 M urea and purified by DEAE-Toyopearl column chromatography via 4 M urea elution; imaging showed entangled knit-like particles. The purified nebulin bound to actin filaments to form loose bundles.

Mouse (Murine) Nebulin (NEB) Interaktionspartner

  1. Nebulin deficiency in adult muscle causes sarcomere defects and muscle-type-dependent changes in trophicity

  2. Nebulin SH3 domain (zeige ITSN1 ELISA Kits) protects against eccentric contraction-induced injury and possibly plays a role in fine-tuning the excitation-contraction coupling mechanism.

  3. Pnn downregulation in skeletal muscle causes a muscular dystrophic phenotype associated with NEB deficiency and the CCD domain is incapable of replacing full length Pnn in terms of functional capacity.

  4. fast skeletal muscle troponin activation increases force at submaximal activation in both wildtype and NEB knockout fiber bundles.

  5. findings show the nebulin-N-WASP complex caused actin nucleation for unbranched actin filament formation from the Z bands without the Arp2 (zeige AICDA ELISA Kits)/3 complex

  6. Results support the view that nebulin plays an important role in skeletal muscle Z-disc width regulation.

  7. The functional roles of nebulin extend beyond thin filament length regulation and include roles in maintaining physiological Z-disk widths and myofibrillar connectivity.

  8. LIM domain and nebulin-repeats to the interaction of Lasp-2 with actin filaments and focal adhesions

  9. These results indicate that, in the absence of nebulin, the attachment probability of the myosin motors to actin is decreased, revealing a direct role for nebulin in promoting strong actomyosin interactions responsible for force and power production.

  10. a means for nebulin-like motifs to participate in the allosteric regulation of striated (zeige NSDHL ELISA Kits) muscle contraction

Cow (Bovine) Nebulin (NEB) Interaktionspartner

  1. Results show that expression of nebulin decreased during the late fattening stage of of hanwoo steers.

Nebulin (NEB) Antigen-Profil

Beschreibung des Gens

This gene encodes nebulin, a giant protein component of the cytoskeletal matrix that coexists with the thick and thin filaments within the sarcomeres of skeletal muscle. In most vertebrates, nebulin accounts for 3 to 4% of the total myofibrillar protein. The encoded protein contains approximately 30-amino acid long modules that can be classified into 7 types and other repeated modules. Protein isoform sizes vary from 600 to 800 kD due to alternative splicing that is tissue-, species-,and developmental stage-specific. Of the 183 exons in the nebulin gene, at least 43 are alternatively spliced, although exons 143 and 144 are not found in the same transcript. Of the several thousand transcript variants predicted for nebulin, the RefSeq Project has decided to create three representative RefSeq records. Mutations in this gene are associated with recessive nemaline myopathy.

Genbezeichner und Symbole assoziert mit Nebulin (NEB) ELISA Kits

  • nebulin (neb) Antikörper
  • nebulin (NEB) Antikörper
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  • AI595938 Antikörper
  • im:6904860 Antikörper
  • im:7001777 Antikörper
  • im:7144538 Antikörper
  • im:7150492 Antikörper
  • NEB Antikörper
  • NEB177D Antikörper
  • NEM2 Antikörper
  • si:ch211-168m7.5 Antikörper
  • wu:fc33b04 Antikörper

Bezeichner auf Proteinebene für Nebulin (NEB) ELISA Kits

nebulin , nemaline myopathy type 2

566928 Danio rerio
695932 Macaca mulatta
374027 Gallus gallus
4703 Homo sapiens
100328572 Oryctolagus cuniculus
17996 Mus musculus
311029 Rattus norvegicus
476149 Canis lupus familiaris
100516703 Sus scrofa
407121 Bos taurus
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