Description:

Size: 100ul

Catalog no.: bs-6479R-A488

Price: 380 EUR

Product details

Gene ID Number

9499

Modification Site

None

Target Antigen

Myotilin

Tested applications

IF(IHC-P)

French translation

anticorps

Clonality

Polyclonal

Modification

Unmodified

Conjugation

Alexa Fluor

Concentration

1ug per 1ul

Excitation emission

499nm/519nm

Conjugated with

ALEXA FLUOR® 488

Crossreactivity

Human, Mouse, Rat

Clone

Polyclonal antibody

Recommended dilutions

IF(IHC-P)(1:50-200)

Purification

Purified by Protein A.

Category

Conjugated Primary Antibodies

Host Organism

Rabbit (Oryctolagus cuniculus)

Also known as

Anti-Myotilin PAb ALEXA FLUOR 488

Specificity

This is a highly specific antibody against Myotilin.

Long name

Myotilin Polyclonal Antibody, ALEXA FLUOR 488 Conjugated

Cross-reactive species details

Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.

Source

This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human Myotilin

Storage conditions

Store this antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Keep refrigerated at 2 to 8 degrees Celcius for up to one year.

Synonyms

57 kDa cytoskeletal protein; LGMD 1; LGMD1; Myofibrillar titin like Ig domains protein; Myofibrillar titin-like Ig domains protein; MYOT; MYOTI_HUMAN; Myotilin; Titin immunoglobulin domain protein; TTID; TTID protein.

Properties

For facs or microscopy Alexa 1 conjugate.Alexa Fluor 488 has the same range to that of fluorescein isothiocyanate (FITC), yet the Anti-Myotilin has a very high photo stability. As a result of this photo stability, it has turned into an antibody for fluorescent microscopy and FACS FLOW cytometry. It is distinguished in the FL1 of a FACS-Calibur or FACScan. Also Alexa Fluor 488 is pH stable.If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies. ALEXA FLUOR they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.

Background of the antigen

Myotilin, a sarcomeric protein that is encoded by the gene mapping to human chromosome 5q31, binds to a-actinin and is localized in the Z-line of myofibrils. Myotilin is expressed in skeletal and cardiac muscle, and it co-localizes with a-actinin in the sarcomeric I-bands where it directly interacts with a-actinin. Defects in the myotilin gene are reported to cause a form of autosomal dominant limb-girdle muscular dystrophy (LGMD). Symptoms of adult onset LGMD are progressive weakness of the hip and shoulder girdles as well as a distinctive dysarthric pattern of speech. The muscle of affected individuals with LGMD shows degeneration of myofibers, variations in fiber size, fiber splitting, centrally located myonuclei and an enhanced number of autophagic vesicles.