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Documentos Principais

HPA008996

Sigma-Aldrich

Anti-JPH1 antibody produced in rabbit

enhanced validation

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

Sinônimo(s):

Anti-JP-1 antibody produced in rabbit, Anti-Junctophilin type 1 antibody produced in rabbit, Anti-Junctophilin-1 antibody produced in rabbit

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About This Item

Código UNSPSC:
12352203
Número do Atlas de Proteínas Humanas:
NACRES:
NA.41

fonte biológica

rabbit

Nível de qualidade

conjugado

unconjugated

forma do anticorpo

affinity isolated antibody

tipo de produto de anticorpo

primary antibodies

clone

polyclonal

linha de produto

Prestige Antibodies® Powered by Atlas Antibodies

forma

buffered aqueous glycerol solution

reatividade de espécies

human

validação aprimorada

orthogonal RNAseq
Learn more about Antibody Enhanced Validation

técnica(s)

immunoblotting: 0.04-0.4 μg/mL
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:200-1:500

sequência de imunogênio

KADAADQAALAARQECDIARAVARELSPDFYQPGPDYVKQRFQEGVDAKENPEEKVPEKPPTPKESPHFYRKGTTPPRSPEASPKHSHSPASSPKPLKKQNPSSGARLNQDKRSVADEQVTAIVNK

nº de adesão UniProt

Condições de expedição

wet ice

temperatura de armazenamento

−20°C

modificação pós-traducional do alvo

unmodified

Informações sobre genes

human ... JPH1(56704)

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Descrição geral

JPH1 (junctophilin 1) is a subtype of the junctional membrane complex protein called JP, which contains three subtypes. This subtype is predominantly expressed in skeletal muscles. It is expressed in the junctional membrane complexes of excitable cells, and exists as a transmembrane protein. This gene is localized to human chromosome 8q21.1.

Imunogênio

Junctophilin-1 recombinant protein epitope signature tag (PrEST)

Aplicação

All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.

The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.

Ações bioquímicas/fisiológicas

Junctophilins span the membrane of intracellular Ca2+ storage vesicles and aids in their interaction with cell-surface membrane, thus, facilitating the formation of junctional membrane complexes. Studies in mice show that this protein plays a crucial role in the formation of triads during skeletal muscle development. JPH1 and GADP-1 are parts of a common pathway, and hence, interdependent. Thus, mutations in GDAP-1 (ganglioside-induced differentiation-associated protein 1) might phenotypically be manifested by JPH1. Therefore, JPH1 is implicated in Charcot-Marie-Tooth (CMT) disease.

Características e benefícios

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.

Ligação

Corresponding Antigen APREST71723

forma física

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

Informações legais

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

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Código de classe de armazenamento

10 - Combustible liquids

Classe de risco de água (WGK)

WGK 1

Ponto de fulgor (°F)

Not applicable

Ponto de fulgor (°C)

Not applicable

Equipamento de proteção individual

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


Certificados de análise (COA)

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M Nishi et al.
Biochemical and biophysical research communications, 273(3), 920-927 (2000-07-13)
Junctophilin (JP) subtypes, namely JP-1, 2, and 3, have been currently identified in excitable cells and constitute a novel family of junctional membrane complex proteins. Our studies have suggested that JPs take part in the formation of junctional membrane complexes
Shinji Komazaki et al.
FEBS letters, 524(1-3), 225-229 (2002-07-24)
Junctophilins (JP-1, JP-2, and JP-3) are transmembrane proteins expressed in the junctional membrane complexes in excitable cells. Both JP-1 and JP-2 are co-expressed in the triads of skeletal muscle, but only JP-2 is expressed in cardiac muscle. We analyzed the
David Pla-Martín et al.
Human molecular genetics, 24(1), 213-229 (2014-08-30)
Mutations in the GDAP1 gene cause different forms of Charcot-Marie-Tooth (CMT) disease, and the primary clinical expression of this disease is markedly variable in the dominant inheritance form (CMT type 2K; CMT2K), in which carriers of the GDAP1 p.R120W mutation
Lucia Golini et al.
The Journal of biological chemistry, 286(51), 43717-43725 (2011-10-25)
Junctophilins (JPs) anchor the endo/sarcoplasmic reticulum to the plasma membrane, thus contributing to the assembly of junctional membrane complexes in striated muscles and neurons. Recent studies have shown that JPs may be also involved in regulating Ca2+ homeostasis. Here, we

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