Key features and details
- Rabbit polyclonal to Myotilin
- Suitable for: IHC-P, WB
- Reacts with: Human
- Isotype: IgG
Product nameAnti-Myotilin antibody
See all Myotilin primary antibodies
DescriptionRabbit polyclonal to Myotilin
Tested applicationsSuitable for: IHC-P, WBmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat, Cow, Pig
Recombinant fragment within Human Myotilin (internal sequence). The exact sequence is proprietary.
Database link: Q9UBF9
- WB: A549 whole cell lysate. IHC: Human hepatoma tissue.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.00
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: PBS, 1% BSA, 20% Glycerol
Concentration information loading...
Our Abpromise guarantee covers the use of ab228763 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||1/100 - 1/1000.|
|WB||1/500 - 1/3000. Predicted molecular weight: 55 kDa.|
FunctionComponent of a complex of multiple actin cross-linking proteins. Involved in the control of myofibril assembly and stability at the Z lines in muscle cells.
Tissue specificityExpressed in skeletal muscle (at protein level). Expressed in skeletal muscle, heart, bone marrow and thyroid gland.
Involvement in diseaseDefects in MYOT are the cause of limb-girdle muscular dystrophy type 1A (LGMD1A) [MIM:159000]. LGMD1A is an autosomal dominant degenerative myopathy with onset within a mean age of 28 years. LGMD1A is characterized by progressive skeletal muscle weakness of the hip and shoulder girdles, later progressing to include distal weakness, as well as a distinctive dysarthric pattern of speech. Affected muscle exhibits disorganization and streaming of the Z-line.
Defects in MYOT are the cause of myopathy myofibrillar myotylin-related (MFM-MYOT) [MIM:609200]. A neuromuscular disorder characterized by progressive skeletal muscle weakness greater distally than proximally, tight heel cords, hyporeflexia, cardiomyopathy and peripheral neuropathy in some patients. Affected muscle exhibits disorganization and streaming of the Z-line, presence of large hyaline structures, excessive accumulation of myotilin and other ectopically expressed proteins and prominent congophilic deposits.
Defects in MYOT are the cause of spheroid body myopathy (SBM) [MIM:182920]. SBM is an autosomal dominant form of myofibrillar myopathy (MFM), characterized by slowly progressing proximal muscle weakness and dysarthric nasal speech. There is no evidence of cardiomyopathy. Muscle biopsy shows spheroid bodies within the type I muscle fibers.
Sequence similaritiesBelongs to the myotilin/palladin family.
Contains 2 Ig-like C2-type (immunoglobulin-like) domains.
Cellular localizationCell membrane > sarcolemma. Cytoplasm > cytoskeleton. Cytoplasm > myofibril > sarcomere > Z line. Sarcomeric, also localized to the sarcolemma. Colocalizes with MYOZ1 at the Z-lines in skeletal muscle.
- Information by UniProt
- 57 kDa cytoskeletal protein antibody
- LGMD 1 antibody
- LGMD1 antibody
Paraffin-embedded human hepatoma tissue stained for Myotilin using ab228763 at 1/500 dilution in immunohistochemical analysis.
Anti-Myotilin antibody (ab228763) at 1/3000 dilution + A549 (human lung carcinoma cell line) whole cell lysate at 30 µg
Developed using the ECL technique.
Performed under reducing conditions.
Predicted band size: 55 kDa
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
ab228763 has not yet been referenced specifically in any publications.