Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Storage bufferpH: 7.30
Preservative: 0.02% Sodium azide
Constituents: 99% Tris buffered saline, 0.5% BSA
Concentration information loading...
PurityImmunogen affinity purified
Purification notesab156953 is purified from Goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Our Abpromise guarantee covers the use of ab156953 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 0.1 - 0.3 µg/ml. Detects a band of approximately 60 kDa (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
Anti-Myotilin antibody (ab156953) at 0.1 µg/ml + Human skeletal muscle lysate at 35 µg
Developed using the ECL technique.
Predicted band size: 55 kDa
Observed band size: 60 kDa why is the actual band size different from the predicted?
ab156953 has not yet been referenced specifically in any publications.