Dysferlin anticorps (Middle Region)

N° du produit ABIN2782235

Cité dans 1 publication. Cet anticorps anti-Dysferlin Polyclonal Lapin (ABIN2782235) détecte spécifiquement Dysferlin dans WB et IHC. L’anticorps est réactif avec des échantillons de Humain, Souris, Rat, Boeuf (Vache), Chien, Cobaye, Lapin, Cheval et Poisson zèbre (Danio rerio).

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

Antigène: Dysferlin (DYSF)

Reactivité: Humain, Souris, Rat, Boeuf (Vache), Chien, Cobaye, Lapin, Cheval, Poisson zèbre (Danio rerio)

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp Dysferlin est non-conjugé

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

Détail du produit anti-Dysferlin anticorps

Épitope: Middle Region

Fonction: DYSF Antibody - 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

Information d'application

Indications d'application: Optimal working dilution should be determined by the investigator.

Commentaires:

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

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 0.5 mg/mL

Buffer: 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: prevent freeze-thaw cycles

Stock: 4 °C,-20 °C

Stockage commentaire: 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.

Références pour anticorps anti-Dysferlin (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." dans: Neuromuscular disorders : NMD, Vol. 17, Issue 11-12, pp. 950-4, (2007) (PubMed).

Détails sur Dysferlin

Antigène: Dysferlin (DYSF)

Autre désignation: DYSF

Sujet:

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

Poids moléculaire: 237kDa

ID gène: 8291

NCBI Accession: NP_003485

UniProt: O75923