SAB1402851
Monoclonal Anti-MYH3 antibody produced in mouse
clone 3H3, purified immunoglobulin, buffered aqueous solution
Synonym(s):
HEMHC, MYHC-EMB, MYHSE1, SMHCE
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About This Item
UNSPSC Code:
12352203
NACRES:
NA.41
biological source
mouse
conjugate
unconjugated
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
3H3, monoclonal
form
buffered aqueous solution
mol wt
antigen ~37 kDa
species reactivity
human
technique(s)
indirect ELISA: suitable
western blot: 1-5 μg/mL
isotype
IgG2aκ
NCBI accession no.
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
Gene Information
human ... MYH3(4621)
General description
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)
Immunogen
MYH3 (NP_002461.1, 2 a.a. ~ 100 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
SSDTEMEVFGIAAPFLRKSEKERIEAQNQPFDAKTYCFVVDSKEEYAKGKIKSSQDGKVTVETEDNRTLVVKPEDVYAMNPPKFDRIEDMAMLTHLNEP
Sequence
SSDTEMEVFGIAAPFLRKSEKERIEAQNQPFDAKTYCFVVDSKEEYAKGKIKSSQDGKVTVETEDNRTLVVKPEDVYAMNPPKFDRIEDMAMLTHLNEP
Application
Monoclonal Anti-MYH3 antibody produced in mouse is suitable for indirect ELISA and western blot applications.
Biochem/physiol Actions
MYH3 (Myosin, heavy chain 3, skeletal muscle, embryonic) plays an important role during the early development of heart. It also plays a role in skeletal development. Mutation in MYH3 causes an autosomal dominant disorder, Multiple pterygium syndrome (MPS), characterized by pterygia, camptodactyly of the hands, vertebral fusions, and scoliosis. MYH3 mutation also causes a severe multiple congenital contracture syndromes, Freeman-Sheldon syndrome (FSS) and Sheldon-Hall syndrome (SHS).
Physical form
Solution in phosphate buffered saline, pH 7.4
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Storage Class Code
13 - Non Combustible Solids
WGK
WGK 1
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Certificates of Analysis (COA)
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Jessica X Chong et al.
American journal of human genetics, 96(5), 841-849 (2015-05-11)
Multiple pterygium syndrome (MPS) is a phenotypically and genetically heterogeneous group of rare Mendelian conditions characterized by multiple pterygia, scoliosis, and congenital contractures of the limbs. MPS typically segregates as an autosomal-recessive disorder, but rare instances of autosomal-dominant transmission have
Catrin Sian Rutland et al.
Development (Cambridge, England), 138(18), 3955-3966 (2011-08-25)
The expression and function of embryonic myosin heavy chain (eMYH) has not been investigated within the early developing heart. This is despite the knowledge that other structural proteins, such as alpha and beta myosin heavy chains and cardiac alpha actin
Reha M Toydemir et al.
Nature genetics, 38(5), 561-565 (2006-04-28)
The genetic basis of most conditions characterized by congenital contractures is largely unknown. Here we show that mutations in the embryonic myosin heavy chain (MYH3) gene cause Freeman-Sheldon syndrome (FSS), one of the most severe multiple congenital contracture (that is
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