Mouse Myotilin (MYOT) ELISA kit; LGMD1; LGMD1A; TTID; titin immunoglobulin domain protein (myotilin) ; myotilin

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of MYOT elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9340853 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the myotilin, ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing MYOT. The ELISA analytical biochemical technique of the MBS9340853 kit is based on MYOT antibody-MYOT antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect MYOT antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, MYOT. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

55,316 Da

myotilin

myotilin; titan-like protein; titin immunoglobulin domain protein; myofibrillar titin-like Ig domains protein

Myotilin

Myo; Ttid??[Similar Products]

MYOTI_MOUSE

MYOT: Component 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. Defects in MYOT are the cause of limb-girdle muscular dystrophy type 1A (LGMD1A). 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 type 3 (MFM3). 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). 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. Belongs to the myotilin/palladin family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Cytoskeletal

Cellular Component: cytoskeleton; membrane; cytoplasm; plasma membrane; Z disc

Molecular Function: actin binding; alpha-actinin binding

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