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Dysferlin antibody [Ham3/17B2]

Cat. No. GTX01941

Host

 Mouse

Clonality

 Monoclonal

Clone Name

 Ham3/17B2

Isotype

 IgG2b

Application

 IHC-P, IHC-Fr

Reactivity

 Human
Package
500 μl ($679)
See all Dysferlin products

APPLICATION

Application Note

*Optimal dilutions/concentrations should be determined by the researcher.
Application Recommended Dilution
IHC-P 1:20–1:40
IHC-Fr 1:20–1:40

Note :

IHC-P
High temperature antigen retrieval using 0.01 M citrate retrieval solution (pH 6.0) is recommended.

IHC-Fr
The recommended fixative is 10% neutral-buffered formalin for paraffin-embedded tissue sections. This antibody requires the sections to be fixed in acetone/methanol at a ratio of 1:1 for 4 minutes at 25ºC prior to incubation with the primary antibody.
Not tested in other applications.

Calculated MW

237 kDa. ( Note )

Product Note

Reactive with the dysferlin molecule in human skeletal muscle. Also present in many non-muscle tissues.

PROPERTIES

Form

Liquid

Buffer

Tissue culture supernatant

Preservative

0.09% Sodium azide

Storage

Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.

Antigen Species

Human

Immunogen

Synthetic peptide containing amino acids 349-366, spanning exons 11 and 12, of the human dysferlin molecule.

Purification

Unpurified

Conjugation

Unconjugated

Note

For laboratory research use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.

Purchasers shall not, and agree not to enable third parties to, analyze, copy, reverse engineer or otherwise attempt to determine the structure or sequence of the product.

TARGET

Synonyms

dysferlin , FER1L1 , LGMD2B , LGMDR2 , MMD1

Cellular Localization

Cell membrane

Background

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. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2008]

Database

Research Area

REFERENCE

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REVIEW

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Package List Price ($)
$ 679