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Rabbit Anti-MYH7B/FITC Conjugated antibody
background:
Myosin heavy chains are ubiquitous Actin-based motor proteins that convert the chemical energy derived from ATP hydrolysis into the mechanical energy that drives diverse motile processes in eukaryotic cells, including cytokinesis, vesicular transport and cellular locomotion. Muscle myosin is a heterohexamer consisting of two myosin heavy chains and two associated nonidentical pairs of myosin light chains. The seven myosin heavy chain isoforms that predominate in mammalian skeletal muscles include two developmental isoforms, MHSLCembryonic (MYH3) and MHSLCperinatal (MYH8); three adult skeletal muscle isoforms, MHC IIa (MYH2), MHC IIb (MYH4) and MHC IIx/d (MYH1); and MHSLC∫/slow (MYH7 or MHSLC∫), which is also expressed in cardiac muscle. Research indicates that mutations of the MYH7 gene causes hypertrophic cardiomyopathy.
Function:
Muscle contraction.
Subunit:
Muscle myosin is a hexameric protein that consists of 2 heavy chain subunits (MHC), 2 alkali light chain subunits (MLC) and 2 regulatory light chain subunits (MLSLC2).
Subcellular Location:
Cytoplasm, myofibril. Note=Thick filaments of the myofibrils.
DISEASE:
Defects in MYH7 are the cause of cardiomyopathy familial hypertrophic type 1 (CMH1) [MIM:192600]. Familial hypertrophic cardiomyopathy is a hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death.
Defects in MYH7 are the cause of myopathy myosin storage (MYOMS) [MIM:608358]. In this disorder, muscle biopsy shows type 1 fiber predominance and increased interstitial fat and connective tissue. Inclusion bodies consisting of the beta cardiac myosin heavy chain are present in the majority of type 1 fibers, but not in type 2 fibers.
Similarity:
Contains 1 IQ domain.
Contains 1 myosin head-like domain.
Database links:
Entrez Gene: 58498 Human
Omim: 612147 Human
SwissProt: Q01449 Human
Unigene: 75636 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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