The Myh3 Antibody (PAC046042) is a powerful tool for researchers studying the Myh3 protein, which is involved in muscle contraction and movement. This polyclonal antibody, produced in rabbits, is highly specific for Myh3 in human samples and is suitable for use in Western blot applications. By binding to the Myh3 protein, this antibody allows for accurate detection and analysis in various cell types, making it an essential tool for studies in muscle biology and muscle-related disorders.Myh3, also known as myosin heavy chain 3, is a key component of muscle fibers and is essential for muscle contraction and movement.
Research on Myh3 is important for understanding muscle development, function, and disease, including conditions such as muscular dystrophy and myopathies. By using the Myh3 Antibody, researchers can gain valuable insights into the role of Myh3 in muscle physiology and pathology, leading to advancements in the field of muscle research and potential therapeutic strategies.
Immunohistochemistry of paraffin-embedded human brain tissue using PACO46042 at dilution of 1:100.
Immunofluorescent analysis of HepG2 cells using PACO46042 at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
Immunohistochemistry of paraffin-embedded human liver tissue using PACO46042 at dilution of 1:100.
Background:
Muscle contraction.
Synonyms:
Myosin-3 (Muscle embryonic myosin heavy chain) (Myosin heavy chain 3) (Myosin heavy chain, fast skeletal muscle, embryonic) (SMHCE), MYH3
UniProt Protein Function:
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; MotorChromosomal Location of Human Ortholog: 17p13.1Cellular Component: sarcomere; muscle myosin complex; cytosolMolecular Function: calmodulin binding; actin filament binding; microfilament motor activity; ATPase activity, coupled; ATP bindingBiological Process: skeletal muscle contraction; muscle development; actin filament-based movement; metabolic process; sarcomere organization; muscle filament sliding; embryonic limb morphogenesisDisease: Arthrogryposis, Distal, Type 8; Arthrogryposis, Distal, Type 2a; Arthrogryposis, Distal, Type 2b
UniProt Protein Details:
NCBI Summary:
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]