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SAB1402851

Sigma-Aldrich

Monoclonal Anti-MYH3 antibody produced in mouse

clone 3H3, purified immunoglobulin, buffered aqueous solution

Sinónimos:

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

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

13 - Non Combustible Solids

wgk_germany

WGK 1

flash_point_f

Not applicable

flash_point_c

Not applicable


Certificados de análisis (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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