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Dysferlin anticorps (Middle Region)

Cet anticorps anti-Dysferlin est un anticorps Lapin Polyclonal détectant Dysferlin dans WB. Adapté pour Humain, Souris, Rat, Boeuf (Vache), Chien, Cobaye, Lapin, Cheval et Poisson zèbre (Danio rerio). Ce Primary Antibody a été cité dans 1 publication.
N° du produit ABIN2782235

Aperçu rapide pour Dysferlin anticorps (Middle Region) (ABIN2782235)

Antigène

Voir toutes Dysferlin (DYSF) Anticorps
Dysferlin (DYSF)

Reactivité

  • 58
  • 24
  • 4
  • 3
  • 3
  • 3
  • 3
  • 2
  • 2
  • 1
  • 1
Humain, Souris, Rat, Boeuf (Vache), Chien, Cobaye, Lapin, Cheval, Poisson zèbre (Danio rerio)

Hôte

  • 52
  • 5
  • 1
Lapin

Clonalité

  • 44
  • 14
Polyclonal

Conjugué

  • 25
  • 4
  • 3
  • 2
  • 2
  • 2
  • 2
  • 2
  • 2
  • 2
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
Cet anticorp Dysferlin est non-conjugé

Application

  • 19
  • 16
  • 15
  • 14
  • 8
  • 6
  • 4
  • 2
  • 2
  • 1
  • 1
Western Blotting (WB)
  • Épitope

    • 16
    • 6
    • 5
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Middle Region

    Séquence

    SRILDESEDT DLPYPPPQRE ANIYMVPQNI KPALQRTAIE ILAWGLRNMK

    Homologie

    Cow: 100%, Dog: 93%, Guinea Pig: 100%, Horse: 100%, Human: 100%, Mouse: 93%, Rabbit: 93%, Rat: 100%, Zebrafish: 92%

    Attributs du produit

    This is a rabbit polyclonal antibody against DYSF. It was validated on Western Blot using a cell lysate as a positive control.

    Purification

    Affinity Purified

    Immunogène

    The immunogen is a synthetic peptide directed towards the middle region of human DYSF
  • Indications d'application

    Optimal working dilutions should be determined experimentally by the investigator.

    Commentaires

    Antigen size: 2080 AA

    Restrictions

    For Research Use only
  • Format

    Liquid

    Concentration

    Lot specific

    Buffer

    Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

    Agent conservateur

    Sodium azide

    Précaution d'utilisation

    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

    Conseil sur la manipulation

    Avoid repeated freeze-thaw cycles.

    Stock

    -20 °C

    Stockage commentaire

    For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
  • Leshinsky-Silver, Argov, Rozenboim, Cohen, Tzofi, Cohen, Wirguin, Dabby, Lev, Sadeh: "Dysferlinopathy in the Jews of the Caucasus: a frequent mutation in the dysferlin gene." dans: Neuromuscular disorders : NMD, Vol. 17, Issue 11-12, pp. 950-4, (2007) (PubMed).

  • Antigène

    Dysferlin (DYSF)

    Autre désignation

    DYSF

    Sujet

    DYSF belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. It is involved in muscle contraction and contains C2 domains that play a role in calcium-mediated membrane fusion events, suggesting that it may be involved in membrane regeneration and repair. In addition, DYSF binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in this gene have been shown to cause autosomal recessive limb girdle muscular dystrophy type 2B (LGMD2B) as well as Miyoshi myopathy.The protein encoded by this gene belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. It is involved in muscle contraction and contains C2 domains that play a role in calcium-mediated membrane fusion events, suggesting that it may be involved in membrane regeneration and repair. In addition, the protein encoded by this gene binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in this gene have been shown to cause autosomal recessive limb girdle muscular dystrophy type 2B (LGMD2B) as well as Miyoshi myopathy. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.
    Alias Symbols: FER1L1, FLJ00175, FLJ90168, LGMD2B, MMD1
    Protein Interaction Partner: UBC, HECW2, HDAC6, CAV3, ANXA2, ANXA1, CAPN3,
    Protein Size: 2080

    Poids moléculaire

    237 kDa

    ID gène

    8291

    NCBI Accession

    NM_003494, NP_003485

    UniProt

    O75923
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