Anti -MYH3 (Myosin-3, Muscle Embryonic Myosin Heavy Chain, Myosin Heavy Chain 3, Myosin Heavy Chain, Fast Skeletal Muscle, Embryonic, SMHCE)

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

Chromosome: 17; NC_000017.10 (10531843..10560626, complement). Location: 17p13.1

Monoclonal

IgG2a,k

3H3

Mouse

Recognizes human MYH3.

Affinity Purified
Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, pH 7.2.

0.5mg/ml (lot specific)

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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.

Antibodies; Abs to Motor Proteins

ELISA (EL/EIA), Western Blot (WB)

Suitable for use in ELISA and Western Blot.
Optimal dilutions to be determined by the researcher.

Western Blot detection against Immunogen (36.63kD).

Detection limit for recombinant GST tagged MYH3 is 0.3ng/ml as a capture antibody.

223,905 Da[Similar Products]

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: Motor; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: 17p13.1

Cellular Component: sarcomere; muscle myosin complex; cytosol

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

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

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

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