Dysferlin antibody (Middle Region)

Catalog No. ABIN2782235

Product Details anti-Dysferlin Antibody

Target: Dysferlin (DYSF)

Binding Specificity: Middle Region

Reactivity: Human, Mouse, Rat, Cow, Dog, Rabbit, Guinea Pig, Horse, Zebrafish (Danio rerio)

Host: Rabbit

Clonality: Polyclonal

Conjugate: This Dysferlin antibody is un-conjugated

Application: Western Blotting (WB)

Sequence: SRILDESEDT DLPYPPPQRE ANIYMVPQNI KPALQRTAIE ILAWGLRNMK

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

Characteristics: 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

Immunogen: The immunogen is a synthetic peptide directed towards the middle region of human DYSF

Application Details

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

Comment:

Antigen size: 2080 AA

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: Lot specific

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

Preservative: Sodium azide

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

Handling Advice: Avoid repeated freeze-thaw cycles.

Storage: -20 °C

Storage Comment: 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.

References for anti-Dysferlin antibody (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).

Target Details for Dysferlin

Target: Dysferlin (DYSF)

Alternative Name: DYSF (DYSF Products)

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

Background: 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

Molecular Weight: 237 kDa

Gene ID: 8291

NCBI Accession: NM_003494, NP_003485

UniProt: O75923