Myosin heavy chain 3; Myosin-3; Myosin heavy chain skeletal muscle embryonic; HEMHC; MYHC-EMB; MYHSE1; SMHCE; myosin, heavy chain 3, skeletal muscle, embryonic

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

Polyclonal

IgG

Rabbit

MYH3 antibody is human, mouse and rat reactive. MYH3 antibody is predicted to not cross-react with other members of the myosin heavy chain family.

MYH3 antibody is affinity chromatography purified via peptide column.

Liquid

1 mg/mL (lot specific)

MYH3 antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to one year.

Small volumes of anti-MYH3 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.

Myosins are actin-based motor proteins that function in the generation of mechanical force in eukaryotic cells (1). MYH3 (myosin, heavy chain, skeletal muscle, embryonic) plays a significant role in skeletal muscle development (2) and is also essential for the proper morphology and function of the developing heart (3). Mutations in this gene have been associated with Freeman-Sheldon syndrome and Sheldon-Hall syndrome (4).

ELISA (EIA), Western Blot (WB), Immunohistochemistry-Paraffin (IHC-P)

MYH3 antibody can be used for detection of MYH3 by Western blot at 1 - 2 ug/ml. Antibody can also be used for immunohistochemistry starting at 5 ug/mL.
Positive Control: Jurkat Cell Lysate MBS151729
Optimal dilutions for each application to be determined by the researcher.

Western blot analysis of MYH3 in fetal human skeletal muscle lysate with MYH3 antibody at 1 μg/ml.

Immunohistochemistry of MYH3 in mouse skeletal muscle tissue with MYH3 antibody at 5 μg/ml.

Predicted: 213 kDa

Observed: 240 kDa

myosin, heavy chain 3, skeletal muscle, embryonic

myosin-3; myosin, skeletal, heavy chain, embryonic 1; myosin heavy chain, fast skeletal muscle, embryonic; myosin, heavy polypeptide 3, skeletal muscle, embryonic

Myosin-3

MYH3_HUMAN

Myosin is a major contractile protein which converts chemical energy into mechanical energy through the hydrolysis of ATP. Myosin is a hexameric protein composed of a pair of myosin heavy chains (MYH) and two pairs of nonidentical light chains. This gene is a member of the MYH family and encodes a protein with an IQ domain and a myosin head-like domain. Mutations in this gene have been associated with two congenital contracture (arthrogryposis) syndromes, Freeman-Sheldon syndrome and Sheldon-Hall syndrome. [provided by RefSeq, Jul 2008]

MYH3: Muscle contraction. Defects in MYH3 are the cause of distal arthrogryposis type 2A (DA2A); also known as Freeman-Sheldon syndrome (FSS). Distal arthrogryposis is a clinically and genetically heterogeneous group of disorders characterized by bone anomalies and joint contractures of the hands and feet, causing medially overlapping fingers, clenched fists, ulnar deviation of fingers, camptodactyly and positional foot deformities. It is a disorder of primary limb malformation without primary neurologic or muscle disease. DA2A is the most severe form of distal arthrogryposis. Affected individuals have contractures of the orofacial muscles, characterized by microstomia with pouting lips, H-shaped dimpling of the chin, deep nasolabial folds, and blepharophimosis. Dysphagia, failure to thrive, growth deficit, and life-threatening respiratory complications (caused by structural anomalies of the oropharynx and upper airways) are frequent. Inheritance is autosomal dominant. Defects in MYH3 are the cause of distal arthrogryposis type 2B (DA2B); also known as Sheldon-Hall syndrome (SHS) or arthrogryposis multiplex congenita distal type 2B (AMCD2B). DA2B is a form of inherited multiple congenital contractures. Affected individuals have vertical talus, ulnar deviation in the hands, severe camptodactyly, and a distinctive face characterized by a triangular shape, prominent nasolabial folds, small mouth and a prominent chin. DA2B is the most common of the distal arthrogryposis syndromes. It is similar to DA2A but the facial contractures are less dramatic.

Protein type: Motility/polarity/chemotaxis; Motor

Chromosomal Location of Human Ortholog: 17p13.1

Cellular Component: sarcomere; muscle myosin complex; cytosol

Molecular Function: microfilament motor activity; actin filament binding; calmodulin binding; ATPase activity, coupled; ATP binding

Biological Process: skeletal muscle contraction; muscle development; actin filament-based movement; metabolic process; sarcomere organization; embryonic limb morphogenesis; muscle filament sliding

Disease: Arthrogryposis, Distal, Type 8; Arthrogryposis, Distal, Type 2a; Arthrogryposis, Distal, Type 2b

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