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Merck
모든 사진(3)

주요 문서

HPA010851

Sigma-Aldrich

Anti-OSTM1 antibody produced in rabbit

enhanced validation

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

동의어(들):

Anti-Osteopetrosis-associated transmembrane protein 1 precursor antibody produced in rabbit

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크기 선택

100 μL
₩917,154

₩917,154


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크기 선택

보기 변경
100 μL
₩917,154

About This Item

UNSPSC 코드:
12352203
인간 단백질 도해서 번호:
NACRES:
NA.41

₩917,154


구입 가능 여부는 고객센터에 문의하십시오.

생물학적 소스

rabbit

Quality Level

결합

unconjugated

항체 형태

affinity isolated antibody

항체 생산 유형

primary antibodies

클론

polyclonal

제품 라인

Prestige Antibodies® Powered by Atlas Antibodies

양식

buffered aqueous glycerol solution

종 반응성

human

향상된 검증

recombinant expression
Learn more about Antibody Enhanced Validation

기술

immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:200-1:500

면역원 서열

REAYKTLSSLYSEMQKMNELENKAEPGTHLCIDVEDAMNITRKLWSRTFNCSVP

UniProt 수납 번호

배송 상태

wet ice

저장 온도

−20°C

타겟 번역 후 변형

unmodified

유전자 정보

human ... OSTM1(28962)

일반 설명

OSTM1 (osteopetrosis associated transmembrane protein 1) is one of the genes associated with Autosomal Recessive Osteopetrosis (ARO) in humans. It was first identified in murine model, as the gene associated with grey-lethal mutant mice. This protein is thought to be an E3 ubiquitin ligase as well as a Gα-interacting protein. It is a type II transmembrane protein, which resides in intracellular compartments. In mice, this gene encodes a protein with predicted 338 amino acids. The region between N- and C- termini of the protein is hydrophobic, and shows slight homology to RING-finger proteins. It also has many putative N-linked glycosylation sites. OSTM1 is expressed in multiple tissues such as, osteoclasts, brain, liver and kidney.

면역원

Osteopetrosis-associated transmembrane protein 1 precursor recombinant protein epitope signature tag (PrEST)

애플리케이션

Anti-OSTM1 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project . Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.

생화학적/생리학적 작용

The exact function of OSTM1 (osteopetrosis associated transmembrane protein 1) gene is not yet known. However, it acts as a β subunit for the protein ClC-7 (Chloride channel 7), as it co-localizes with ClC-7 in lysosomes and late endosomes in multiple tissues, as well as in the bone-resorbing osteoclasts, in their ruffled borders. This gene is essential for the maturation and normal function of osteocytes and melanocytes. In humans, mutations in this gene lead to autosomal recessive osteopetrosis (ARO). ARO is a rare hereditary disorder, which is a result of oscteoclast resorption failure, and is characterized by bone marrow failure, increased bone density and fractures. OSTM1 is also responsible for normal neural development, and mutations in this gene lead to ARO with severe neuronopathy. This phenotype is characterized by malformations in the central nervous system, blindness, seizures, and deafness.

특징 및 장점

Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:
  • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
  • Protein array of 364 human recombinant protein fragments.

결합

Corresponding Antigen APREST71674

물리적 형태

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide

법적 정보

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

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

10 - Combustible liquids

WGK

WGK 1

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable

개인 보호 장비

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


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문서 라이브러리 방문

Claus-Eric Ott et al.
Bone, 55(2), 292-297 (2013-05-21)
Autosomal recessive osteopetrosis (ARO, MIM 259700) is a genetically heterogeneous rare skeletal disorder characterized by failure of osteoclast resorption leading to pathologically increased bone density, bone marrow failure, and fractures. In the neuronopathic form neurological complications are especially severe and
Nader Chalhoub et al.
Nature medicine, 9(4), 399-406 (2003-03-11)
The spontaneous mouse grey-lethal (gl) mutation is responsible for a coat color defect and for the development of the most severe autosomal recessive form of osteopetrosis. Using a positional cloning approach, we have mapped and isolated the gl locus from
Philipp F Lange et al.
Nature, 440(7081), 220-223 (2006-03-10)
Mutations in ClC-7, a late endosomal/lysosomal member of the CLC family of chloride channels and transporters, cause osteopetrosis and lysosomal storage disease in humans and mice. Severe osteopetrosis is also observed with mutations in the OSTM1 gene, which encodes a
Alessandra Pangrazio et al.
Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research, 21(7), 1098-1105 (2006-07-04)
We report three novel osteopetrosis patients with OSTM1 mutations and review two that have been previously described. Our analysis suggests that OSTM1 defines a new subset of patients with severe central nervous system involvement. This defect is also present in
Erika A Bosman et al.
Mammalian genome : official journal of the International Mammalian Genome Society, 24(1-2), 44-53 (2012-11-20)
Large-scale N-ethyl-N-nitrosourea (ENU) mutagenesis has provided many rodent models for human disease. Here we describe the initial characterization and mapping of a recessive mutation that leads to degeneration of the incisors, failure of molars to erupt, a grey coat colour

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