Dysferlin antibody (Middle Region)

Catalog No. ABIN502635

Cited in 1 publication. The Rabbit Polyclonal anti-Dysferlin antibody (ABIN502635) specifically detects Dysferlin in WB and IHC. The antibody is reactive with Human, Mouse, Rat, Cow, Dog, Guinea Pig, Rabbit, Horse and Zebrafish (Danio rerio) samples.

Quick Overview for Dysferlin antibody (Middle Region) (ABIN502635)

Target: Dysferlin (DYSF)

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

Host: Rabbit

Clonality: Polyclonal

Conjugate: This Dysferlin antibody is un-conjugated

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

Product Details anti-Dysferlin Antibody

Binding Specificity: Middle Region

Purpose: DYSF Antibody - middle region

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 dilution should be determined by the investigator.

Comment:

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

Handling

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.

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: prevent freeze-thaw cycles

Storage: 4 °C,-20 °C

Storage Comment: 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.

References for anti-Dysferlin antibody (ABIN502635)

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

Background:

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

Molecular Weight: 237kDa

Gene ID: 8291

NCBI Accession: NP_003485

UniProt: O75923