Dysferlin Antikörper (Middle Region)

Produktnummer ABIN2782235

Produktdetails anti-Dysferlin Antikörper

Target: Dysferlin (DYSF)

Bindungsspezifität: Middle Region

Reaktivität: Human, Maus, Ratte, Hund, Rind (Kuh), Kaninchen, Meerschweinchen, Pferd, Zebrafisch (Danio rerio)

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser Dysferlin Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

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

Anwendungsinformationen

Applikationshinweise: Optimal working dilutions should be determined experimentally by the investigator.

Kommentare:

Antigen size: 2080 AA

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

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.

Referenzen für anti-Dysferlin Antikörper (ABIN2782235)

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).

Details zu Dysferlin

Target: Dysferlin (DYSF)

Andere Bezeichnung: DYSF (DYSF Produkte)

Synonyme: DYSF antikoerper, fb73b05 antikoerper, wu:fb73b05 antikoerper, si:rp71-50c18.1 antikoerper, DKFZp459E1226 antikoerper, 2310004N10Rik antikoerper, AI604795 antikoerper, D6Pas3 antikoerper, mFLJ00175 antikoerper, FER1L1 antikoerper, LGMD2B antikoerper, MMD1 antikoerper, dysferlin antikoerper, dysferlin, limb girdle muscular dystrophy 2B (autosomal recessive) antikoerper, myoferlin antikoerper, DYSF antikoerper, dysf antikoerper, LOC589501 antikoerper, Dysf antikoerper

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