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Dysferlin Antikörper (Middle Region)

Dieses Anti-Dysferlin-Antikörper ist ein Kaninchen Polyklonal-Antikörper zur Detektion von Dysferlin in WB. Geeignet für Human, Maus, Ratte, Rind (Kuh), Hund, Meerschweinchen, Kaninchen, Pferd und Zebrafisch (Danio rerio). Dieses Primary Antibody wurde in 1 Publikation zitiert.
Produktnummer ABIN2782235

Kurzübersicht für Dysferlin Antikörper (Middle Region) (ABIN2782235)

Target

Alle Dysferlin (DYSF) Antikörper anzeigen
Dysferlin (DYSF)

Reaktivität

  • 59
  • 24
  • 4
  • 3
  • 3
  • 3
  • 3
  • 2
  • 2
  • 1
  • 1
Human, Maus, Ratte, Rind (Kuh), Hund, Meerschweinchen, Kaninchen, Pferd, Zebrafisch (Danio rerio)

Wirt

  • 53
  • 5
  • 1
Kaninchen

Klonalität

  • 46
  • 13
Polyklonal

Konjugat

  • 26
  • 4
  • 3
  • 2
  • 2
  • 2
  • 2
  • 2
  • 2
  • 2
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
Dieser Dysferlin Antikörper ist unkonjugiert

Applikation

  • 19
  • 17
  • 15
  • 15
  • 9
  • 7
  • 5
  • 2
  • 2
  • 1
  • 1
Western Blotting (WB)
  • Bindungsspezifität

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

    Sequenz

    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%

    Produktmerkmale

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

    Aufreinigung

    Affinity Purified

    Immunogen

    The immunogen is a synthetic peptide directed towards the middle region of human DYSF
  • Applikationshinweise

    Optimal working dilutions should be determined experimentally by the investigator.

    Kommentare

    Antigen size: 2080 AA

    Beschränkungen

    Nur für Forschungszwecke einsetzbar
  • Format

    Liquid

    Konzentration

    Lot specific

    Buffer

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

    Konservierungsmittel

    Sodium azide

    Vorsichtsmaßnahmen

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

    Handhabung

    Avoid repeated freeze-thaw cycles.

    Lagerung

    -20 °C

    Informationen zur Lagerung

    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." in: Neuromuscular disorders : NMD, Vol. 17, Issue 11-12, pp. 950-4, (2007) (PubMed).

  • Target

    Dysferlin (DYSF)

    Andere Bezeichnung

    DYSF

    Hintergrund

    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

    Molekulargewicht

    237 kDa

    Gen-ID

    8291

    NCBI Accession

    NM_003494, NP_003485

    UniProt

    O75923
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