HPA017071
Anti-DYSF antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
Sinônimo(s):
Anti-Dysferlin, Anti-Dystrophy-associated fer-1-like protein, Anti-Fer-1-like protein 1
Faça loginpara ver os preços organizacionais e de contrato
About This Item
Produtos recomendados
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
Formulário
buffered aqueous glycerol solution
reatividade de espécies
human
validação aprimorada
orthogonal RNAseq
independent
Learn more about Antibody Enhanced Validation
técnica(s)
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:200-1:500
sequência de imunogênio
IVVELYDHDTYGADEFMGRCICQPSLERMPRLAWFPLTRGSQPSGELLASFELIQREKPAIHHIPGFEVQETSRILDESEDTDLPYPPPQREANIYMVPQNIKPALQRTAIEILAWG
nº de adesão UniProt
Condições de expedição
wet ice
temperatura de armazenamento
−20°C
modificação pós-traducional do alvo
unmodified
Informações sobre genes
human ... DYSF(8291)
Imunogênio
Dysferlin 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.
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
DYSF is identified as a component of a putative muscle-specific repair complex involved in the plasma membrane or muscle membrane repair machinery. In addition to patch repair, it also performs in the membrane trafficking via dysferlin-related signaling pathways in muscle cell. During repatching, it provides cell membrane scaffold like structure, which gives structural stability to muscle cells. Deficiency of dysferlin disrupts sarcolemmal membrane repair, termed as limb-girdle muscular dystrophies type 2B. Mutation in the gene also causes miyoshi myopathy.
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:
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 APREST71976
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
Exoneração de responsabilidade
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.
Não está encontrando o produto certo?
Experimente o nosso Ferramenta de seleção de produtos.
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)
Escolha uma das versões mais recentes:
Certificados de análise (COA)
Lot/Batch Number
Não está vendo a versão correta?
Se precisar de uma versão específica, você pode procurar um certificado específico pelo número do lote ou da remessa.
Já possui este produto?
Encontre a documentação dos produtos que você adquiriu recentemente na biblioteca de documentos.
Hiroya Ono et al.
Molecular therapy : the journal of the American Society of Gene Therapy, 28(4), 1133-1153 (2020-02-24)
Mutations in dysferlin are responsible for a group of progressive, recessively inherited muscular dystrophies known as dysferlinopathies. Using recombinant proteins and affinity purification methods combined with liquid chromatography-tandem mass spectrometry (LC-MS/MS), we found that AMP-activated protein kinase (AMPK)γ1 was bound
Rumiko Izumi et al.
Human mutation, 41(9), 1540-1554 (2020-05-14)
Dysferlinopathy is a group of autosomal recessive muscular dystrophies caused by variants in the dysferlin gene (DYSF), with variable proximal and distal muscle involvement. We performed DYSF gene analyses of 200 cases suspected of having dysferlinopathy (Cohort 1), and identified
Louise Glover et al.
Traffic (Copenhagen, Denmark), 8(7), 785-794 (2007-06-06)
The muscular dystrophies are a heterogeneous group of inherited disorders, defined by progressive muscle weakness and atrophy. Following the discovery of dystrophin, remarkable progress has been made in defining the molecular properties of proteins involved in the various dystrophies. This
S H Laval et al.
Neuropathology and applied neurobiology, 30(2), 91-105 (2004-03-27)
The limb-girdle muscular dystrophies are a diverse group of muscle-wasting disorders characteristically affecting the large muscles of the pelvic and shoulder girdles. Molecular genetic analyses have demonstrated causative mutations in the genes encoding a disparate collection of proteins involved in
Dimple Bansal et al.
Nature, 423(6936), 168-172 (2003-05-09)
Muscular dystrophy includes a diverse group of inherited muscle diseases characterized by wasting and weakness of skeletal muscle. Mutations in dysferlin are linked to two clinically distinct muscle diseases, limb-girdle muscular dystrophy type 2B and Miyoshi myopathy, but the mechanism
Nossa equipe de cientistas tem experiência em todas as áreas de pesquisa, incluindo Life Sciences, ciência de materiais, síntese química, cromatografia, química analítica e muitas outras.
Entre em contato com a assistência técnica