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See also: Nebulin Nebulous Nebula Nebulizer Nebulization Nebulisers Nebulized Nebulised Neblina Nebulae Nebular Nebuchadnezzar Nebulosa Nebulously Nebulosity

1. Nebulin, encoded by NEB, is a giant skeletal muscle protein of about 6669 amino acids which forms an integral part of the sarcomeric thin filament. In recent years, the nebula around this protein has been largely lifted resulting in the discovery that Nebulin

Nebulin, Neb, Nebula

2. Nebulin plays roles in specifying thin filament length, stabilizing and activating the filaments, as well as in ensuring force generation and efficiency of contraction (354–356). From: Emery and Rimoin's Principles and Practice of Medical Genetics, 2013

Nebulin

3. Nebulin is a giant protein (600-900 kDa) that is also part of the thin filaments.

Nebulin

4. Nebulette, the cardiac isoform of the actin-binding protein Nebulin, localizes to the Z-disc in cardiomyocytes, where it binds to a variety of Z-disc proteins, such as actin, α-actinin, CapZ, titin, and myopalladin (99)

Nebulette, Nebulin

5. Nebulin is a giant filamentous protein specific for vertebrate skeletal muscles

Nebulin

6. The correlation of its size to thin filament lengths in vertebrates suggests that Nebulin may function as a molecular ruler to determine thin filament length

Nebulin

7. We have isolated a full-length cDNA of 20.8 kb encoding human Nebulin and determined its sequence.

Nebulin

8. Nebulin's SH3 domain is highly related in sequence to the SH3 domains in yeast actin binding protein ABP-1 and to the src substrate p80/85 in chicken, both proteins which are involved in regulating actin assembly of the cytoskeleton in non-muscle cells

Nebulin, Non

9. Study of Nebulins terminal sequences is likely to reveal how integration of the Nebulin

Nebulins, Nebulin

10. Nebulin is a giant actin-binding protein in skeletal muscle which localizes along most of the length of the thin filament. Genetic alterations or reduction in the expression level of Nebulin are accompanied by dramatic loss in muscle force, resulting in muscle weakness and severe skeletal muscle myopathy.

Nebulin

11. The NEB gene provides instructions for making a protein called Nebulin. This protein plays an important role in skeletal muscles, which are muscles used for movement

Neb, Nebulin

12. Within skeletal muscle cells, Nebulin is found in structures called sarcomeres

Nebulin

13. Cd11933, SH3_Nebulin_C, 1 hit: InterPro i: View protein in InterPro IPR013998, Nebulin-like IPR000900, Nebulin_repeat IPR035629, Nebulin_SH3 IPR036028, SH3-like_dom_sf IPR001452, SH3_domain: Pfam i: View protein in Pfam PF00880, Nebulin, 81 hits PF14604, SH3_9, 1 hit: PRINTS i: PR00510, Nebulin: SMART i

Nebulin

14. Nebulin is a giant filamentous protein found in skeletal muscle where it spans from the sarcomere's Z-disk to near the tip of the thin filament. The long-term goal of this proposal is to gain a mechanistic understanding of the roles of Nebulin in skeletal muscle health and disease.

Nebulin, Near

15. The main source of the Nebulin length variation in different species is the number of actin- and tropomyosin-binding super-repeat domains

Nebulin, Number

16. Nebulin plays a role in numerous cellular processes including regulation of muscle contraction, Z-disc formation, and myofibril organization and assembly.

Nebulin, Numerous

17. 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.

Nebulin

18. Nebulin, encoded by NEB, is a giant skeletal muscle protein of about 6669 amino acids which forms an integral part of the sarcomeric thin filament

Nebulin, Neb

19. Nebulin is a giant 600‐ to 900‐kDa filamentous protein that is an integral component of the skeletal muscle thin filament

Nebulin

20. Its functions have remained largely nebulous because of its large size and the difficulty in extracting Nebulin in a native state from muscle.

Nebulous, Nebulin, Native

21. Nebulin’s control is less tight in slow muscle types where a distal Nebulin-free thin-filament segment exists, the length of which was found to be regulated by leiomodin-2 (Lmod2)

Nebulin

22. We propose that strict length control by Nebulin promotes high-speed shortening and that dual-regulation by Nebulin/Lmod2 enhances contraction efficiency.

Nebulin

23. The Nebulin (NEB) gene is the most commonly mutated and is thought to account for approximately 50% of genetically diagnosed cases of NM

Nebulin, Neb, Nm

24. It is composed by 183 exons of which at least 17 have been shown to be alternatively spliced, giving rise to several different Nebulin isoforms in skeletal muscle .

Nebulin

25. Nebulin is a giant protein component of the cytoskeletal matrix that coexists with the thick and thin filaments within the sarcomeres of skeletal muscle.

Nebulin

26. We have previously described 18 different mutations in the last 42 exons of the Nebulin gene (NEB) in 18 families with NM.

Nebulin, Neb, Nm

27. Nebulin is a giant 600‐ to 900‐kDa filamentous protein that is an integral component of the skeletal muscle thin filament

Nebulin

28. Its functions have remained largely nebulous because of its large size and the difficulty in extracting Nebulin in a native state from muscle.

Nebulous, Nebulin, Native

29. Nebulin is Involved in Specifying Thin Filament Length Skeletal muscle produces efficient contractile force because they contain thin and thick filaments of well-defined lengths that are organized into regular, symmetric arrays that interdigitate.

Nebulin

30. Our Nebulin Peptides and Nebulin Proteins can be used in a variety of model species: Human

Nebulin

31. Use the list below to choose the Nebulin Peptide and Nebulin Protein which is most appropriate for your research; you can click on each one to view full technical details, images, references, reviews and …

Nebulin

32. Nemaline myopathy (NM) caused by mutations in the gene encoding Nebulin (NEB) accounts for at least 50% of all NM cases worldwide, representing a significant disease burden

Nemaline, Nm, Nebulin, Neb

33. Nebulin is a giant (600 to 900 kDa) filamentous protein constituting part of the skeletal muscle thin filament

Nebulin

34. Around 90% of the primary structure of Nebulin is composed of approximately 35-residue α-helical domains, which form super repeats that bind actin with high affinity

Nebulin

35. These mAbs, prepared against a fragment of human skeletal muscle Nebulin located near the carboxyl terminus, detect a 107-kD pr … A 107-kD protein has been identified in primary cultures of chicken embryonic cardiomyocytes by immunoprecipitations with …

Nebulin, Near

36. Looking for online definition of Nebulin or what Nebulin stands for? Nebulin is listed in the World's largest and most authoritative dictionary database of abbreviations and acronyms The Free Dictionary

Nebulin

37. Nebulin is a critical thin filament-binding protein that spans from the Z-disk of the skeletal muscle sarcomere to near the pointed end of the thin filament

Nebulin, Near

38. Nebulin Add Nebulin protein, human Add Nebulin protein, mouse Add Nebulin protein, rat Add Pharm Action Registry Number 02X6KNJ5EE CAS Type 1 Name NLM Classification # Previous Indexing See Also Consider Also Public MeSH Note Online Note History Note Entry Combination Heading Mapped to *Muscle Proteins Frequency

Nebulin, Number, Name, Nlm, Note

39. Recessive Nebulin (NEB) mutations are a common cause of nemaline myopathy (NM), typically characterized by generalized weakness of early-onset and nemaline rods on muscle biopsy

Nebulin, Neb, Nemaline, Nm

40. Nebulin is a giant filamentous protein that is coextensive with the actin filaments of the skeletal muscle sarcomere

Nebulin

41. Nebulin mutations are the main cause of nemaline myopathy (NEM), with typical adult patients having low expression of Nebulin, yet the roles of Nebulin in adult muscle remain poorly understood.

Nebulin, Nemaline, Nem

42. Mutations in the Nebulin gene (NEB) are the most common cause of autosomal recessive nemaline myopathy (NEM)

Nebulin, Neb, Nemaline, Nem

43. J:291849 Lindqvist J, et al., Triggering typical nemaline myopathy with compound heterozygous Nebulin mutations reveals myofilament structural changes as pathomechanism

Nemaline, Nebulin

44. Nebulin is a giant (600–900 kDa) muscle protein expressed predominantly in the thin filaments of striated muscle

Nebulin

45. Nebulin has a highly repetitive protein …

Nebulin

46. For example, Nebulin is capable of interacting with the pointed end-capping protein Tropomodulin 1 via its first three N-terminal Nebulin repeats (McElhinny et al., 2001)

Nebulin

47. In addition, the SH3 domain of Nebulin has been shown to interact with the barbed end capping protein CapZ (Pappas et al., 2008)

Nebulin

48. In this way, Nebulin is able to cap actin

Nebulin

49. Nebulin is a giant myofibrillar protein found within the sarcomeres of skeletal muscle.Over 95% of the Nebulin protein consists of 185 copies of a 35- to 40-amino acid module

Nebulin

50. Most of the modules are arranged into a higher order repeating sequence termed a 'Nebulin super repeat.'

Nebulin

51. Nebulin is a large myofibrillar protein that binds filamentous actin (F-actin) and may also associate with tropomyosin and tropomodulin while anchoring to the z-line of sarcomeres (A)

Nebulin

52. The largest NEB transcript is predicted to encode a 5907 amino acid long protein consisting of approximately 165 Nebulin motifs (NEBU)

Neb, Nebulin, Nebu

53. Mini-Nebulin can displace endogenous Nebulin and does not restrict actin filament lengths or Tmod1 localization

Nebulin, Not

54. (A) Chick skeletal myocytes cotransfected with control siRNA alone or control siRNA plus mCherry–mini-Nebulin were triple stained with fluorescently conjugated phalloidin (which labels F-actin; b, f, and j) and antibodies to N

Nebulin

55. Nebulin Antibodies 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

Nebulin

56. In most vertebrates, Nebulin accounts for 3 to 4% of the total myofibrillar protein.

Nebulin

57. Background Nebulin is a critical thin filament-binding protein that spans from the Z-disk of the skeletal muscle sarcomere to near the pointed end of the thin filament

Nebulin, Near

58. Nebulin is a giant sarcomeric protein that spans along the actin filament in skeletal muscle, from the Z-disk to near the thin filament pointed end

Nebulin, Near

59. Mutations in Nebulin cause muscle weakness in nemaline myopathy patients, suggesting that Nebulin plays impor-tant roles in force generation, yet little is known about Nebulin’s

Nebulin, Nemaline

60. Nebulin is a high molecular weight protein (500-800 kD), localized at the N2-line of skeletal muscle myofibrils

Nebulin

61. NEB (Nebulin) is a Protein Coding gene

Neb, Nebulin

62. We found that IGF-1–induced phosphatidylinositol 3-kinase–Akt signaling formed a complex of Nebulin and N-WASP at the Z bands …

Nebulin

63. Titin and Nebulin content and the ratio of titin to Nebulin before and after exercise were compared using a paired t-test

Nebulin

64. Pearson product cor-relation was used to compare the change in titin to the change in Nebulin from before to after exercise

Nebulin

65. Pour me contacter : Nebulin[email protected]

Nebulinecrea

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Dictionary

NEBULIN

    Frequently Asked Questions

    What does nebulin mean?

    neb·ul·in. A large protein, constituting about 3% of skeletal muscle protein; may aid in the organization of actin filaments as well as in actin polymerization. [L. nebula, mist, fog, fr. nebulin. A large protein (600 to 800 kD in mass) found in the thin filaments of contractile muscles, where it binds to actin.

    What is nebulin binding protein?

    Nebulin is an actin-binding protein which is localized to the thin filament of the sarcomeres in skeletal muscle. It is a very large protein (600–900 kDa) and binds as many as 200 actin monomers.

    Is nebulin truly a component of the thin filament?

    Nebulin is an actin -binding protein which is localized to the thin filament of the sarcomeres in skeletal muscle. It is a very large protein (600–900 kDa) and binds as many as 200 actin monomers. Because its length is proportional to thin filament length, it is believed that nebulin acts as a thin filament "ruler"...

    What is the role of nebulin in skeletal muscle?

    Nebulin regulates the assembly and lengths of the thin filaments in striated muscle. New insights into the structural roles of nebulin in skeletal muscle. Detection of giant myofibrillar proteins connectin and nebulin in fish meat by electrophoresis in 3-5 gradient sodium dodecyl sulfate polyacrylamide slab gels.

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