MYOT, Polyclonal Antibody

MFM3; TTID; TTOD; LGMD1; LGMD1A

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

Polyclonal

IgG

Rabbit

Affinity Purification

1mg/ml (lot specific)

Store at -20 degree C (regular) or -80 degree C (long term). Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Small volumes of anti-MYOT antibody 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.

This gene encodes a cystoskeletal protein which plays a significant role in the stability of thin filaments during muscle contraction. This protein binds F-actin, crosslinks actin filaments, and prevents latrunculin A-induced filament disassembly. Mutations in this gene have been associated with limb-girdle muscular dystrophy and myofibrillar myopathies. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined.

Polyclonal

Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

WB: 1:500 - 1:2000
IHC: 1:50 - 1:200

Western blot analysis of extracts of various cell lines, using MYOT antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Enhanced Kit.
Exposure time: 90s.

Immunohistochemistry of paraffin-embedded mouse heart using MYOT antibody at dilution of 1:100 (40x lens).

Immunofluorescence analysis of U2OS cells using MYOT antibody. Blue: DAPI for nuclear staining.

498

myotilin

myotilin; 57 kDa cytoskeletal protein; myofibrillar titin-like Ig domains protein; titin immunoglobulin domain protein (myotilin)

Myotilin

TTID  [Similar Products]

MYOTI_HUMAN

This gene encodes a cystoskeletal protein which plays a significant role in the stability of thin filaments during muscle contraction. This protein binds F-actin, crosslinks actin filaments, and prevents latrunculin A-induced filament disassembly. Mutations in this gene have been associated with limb-girdle muscular dystrophy and myofibrillar myopathies. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined.[provided by RefSeq, Oct 2008]

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

Chromosomal Location of Human Ortholog: 5q31

Cellular Component: Z disc; sarcolemma; actin cytoskeleton

Molecular Function: protein binding; structural constituent of muscle; alpha-actinin binding; actin binding

Biological Process: muscle contraction

Disease: Myopathy, Spheroid Body; Muscular Dystrophy, Limb-girdle, Type 1a; Myopathy, Myofibrillar, 3

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