antibody

MYH3 Antibody

MBS153499

0.1 mg

345 USD

polyclonal

rabbit

nonconjugated

human, mouse, rat

western blot, ELISA, enzyme immunoassay, immunohistochemistry - paraffin section

Antibody

MYH3 Antibody

MYH3

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

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

MYH3

MYH3; MYH3; HEMHC; SMHCE; MYHSE1; MYHC-EMB

MYH3_HUMAN

Polyclonal

IgG

Rabbit

Human, Mouse, Rat

1940

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

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

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.

Conjugate: Unconjugated. Immunogen: MYH3 antibody was raised against a 19 amino acid peptide near the amino terminus of human MYH3. Buffer: MYH3 antibody is supplied in PBS containing 0.02% sodium azide.

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).

98986453

NP_002461

NM_002470.3

P11055

Predicted: 213 kDa. Observed: 240 kDa

Membrane Trafficking Pathway (106160); Muscle Contraction Pathway (106261); Striated Muscle Contraction Pathway (198903); Striated Muscle Contraction Pathway (106262); Tight Junction Pathway (83071); Tight Junction Pathway (482); Translocation Of GLUT4 To The Plasma Membrane Pathway (685554)

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

0.1 mg

345 USD