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Documenti fondamentali

SAB1402851

Sigma-Aldrich

Monoclonal Anti-MYH3 antibody produced in mouse

clone 3H3, purified immunoglobulin, buffered aqueous solution

Sinonimo/i:

HEMHC, MYHC-EMB, MYHSE1, SMHCE

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About This Item

Codice UNSPSC:
12352203
NACRES:
NA.41

Origine biologica

mouse

Coniugato

unconjugated

Forma dell’anticorpo

purified immunoglobulin

Tipo di anticorpo

primary antibodies

Clone

3H3, monoclonal

Stato

buffered aqueous solution

PM

antigen ~37 kDa

Reattività contro le specie

human

tecniche

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

Isotipo

IgG2aκ

N° accesso NCBI

N° accesso UniProt

Condizioni di spedizione

dry ice

Temperatura di conservazione

−20°C

Informazioni sul gene

human ... MYH3(4621)

Descrizione generale

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)

Immunogeno

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

Applicazioni

Monoclonal Anti-MYH3 antibody produced in mouse is suitable for indirect ELISA and western blot applications.

Azioni biochim/fisiol

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).

Stato fisico

Solution in phosphate buffered saline, pH 7.4

Esclusione di responsabilità

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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Codice della classe di stoccaggio

13 - Non Combustible Solids

Classe di pericolosità dell'acqua (WGK)

WGK 1

Punto d’infiammabilità (°F)

Not applicable

Punto d’infiammabilità (°C)

Not applicable


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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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