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SAB1402851

Monoclonal Anti-MYH3 antibody produced in mouse

Name: Monoclonal Anti-MYH3 antibody produced in mouse
Brand: SIGMA

clone 3H3, purified immunoglobulin, buffered aqueous solution

Synonyme(s) :

HEMHC, MYHC-EMB, MYHSE1, SMHCE

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About This Item

Code UNSPSC :

12352203

Nomenclature NACRES :

NA.41

Source biologique

mouse

Conjugué

unconjugated

Forme d'anticorps

purified immunoglobulin

Type de produit anticorps

primary antibodies

Clone

3H3, monoclonal

Forme

buffered aqueous solution

Poids mol.

antigen ~37 kDa

Espèces réactives

human

Technique(s)

indirect ELISA: suitable
western blot: 1-5 μg/mL

Isotype

IgG2aκ

Numéro d'accès NCBI

NM_002470

Numéro d'accès UniProt

P11055

Conditions d'expédition

dry ice

Température de stockage

−20°C

Informations sur le gène

human ... MYH3(4621)

Description générale

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)

Immunogène

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.

Actions biochimiques/physiologiques

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).

Forme physique

Solution in phosphate buffered saline, pH 7.4

Clause de non-responsabilité

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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Code de la classe de stockage

13 - Non Combustible Solids

Classe de danger pour l'eau (WGK)

WGK 1

Point d'éclair (°F)

Not applicable

Point d'éclair (°C)

Not applicable

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Certificats d'analyse (COA)

Lot/Batch Number

08228-3H3

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Autosomal-Dominant Multiple Pterygium Syndrome Is Caused by Mutations in MYH3.

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

Knockdown of embryonic myosin heavy chain reveals an essential role in the morphology and function of the developing heart.

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

Mutations in embryonic myosin heavy chain (MYH3) cause Freeman-Sheldon syndrome and Sheldon-Hall syndrome.

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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