Dysferlin Antikörper (Middle Region)

Produktnummer ABIN2782235

Zitiert in 1 Publikation. Der Kaninchen Polyklonal anti-Dysferlin Antikörper (ABIN2782235) detektiert spezifisch Dysferlin in WB und IHC. Dieser Antikörper reagiert spezifisch mit Proben aus Human, Maus, Ratte, Rind (Kuh), Hund, Meerschweinchen, Kaninchen, Pferd und Zebrafisch (Danio rerio).

Kurzübersicht für Dysferlin Antikörper (Middle Region) (ABIN2782235)

Target: Dysferlin (DYSF)

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

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser Dysferlin Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Immunohistochemistry (IHC)

Produktdetails anti-Dysferlin Antikörper

Bindungsspezifität: Middle Region

Verwendungszweck: DYSF Antibody - 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

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Kommentare:

We also sell a specific blocking peptide that can be used in combination with this antibody. You can find the blocking peptide under AAP45478-100UG

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 0.5 mg/mL

Buffer: 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: prevent freeze-thaw cycles

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: For short term use, store at 2-8C 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

Hintergrund:

Background Information: 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.

Gene Name: Dysferlin, limb girdle muscular dystrophy 2B (autosomal recessive)

Alternative Symbols: MMD1, FER1L1, LGMD2B, LGMDR2

Protein Name: Dysferlin

Molekulargewicht: 237kDa

Gen-ID: 8291

NCBI Accession: NP_003485

UniProt: O75923