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Merck
모든 사진(2)

주요 문서

SAB1402851

Sigma-Aldrich

Monoclonal Anti-MYH3 antibody produced in mouse

clone 3H3, purified immunoglobulin, buffered aqueous solution

동의어(들):

HEMHC, MYHC-EMB, MYHSE1, SMHCE

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About This Item

UNSPSC 코드:
12352203
NACRES:
NA.41

생물학적 소스

mouse

결합

unconjugated

항체 형태

purified immunoglobulin

항체 생산 유형

primary antibodies

클론

3H3, monoclonal

양식

buffered aqueous solution

분자량

antigen ~37 kDa

종 반응성

human

기술

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

동형

IgG2aκ

NCBI 수납 번호

UniProt 수납 번호

배송 상태

dry ice

저장 온도

−20°C

유전자 정보

human ... MYH3(4621)

일반 설명

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)

면역원

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

애플리케이션

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

생화학적/생리학적 작용

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).

물리적 형태

Solution in phosphate buffered saline, pH 7.4

면책조항

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


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문서 라이브러리 방문

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
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
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

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