Dysferlin Antikörper

Produktnummer ABIN265388

Produktdetails anti-Dysferlin Antikörper

Target: Dysferlin (DYSF)

Reaktivität: Human, Maus

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser Dysferlin Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Immunofluorescence (IF), Enzyme Immunoassay (EIA)

Spezifität: This antibody detects endogenous levels of DP-1/TFDP1 protein (region surrounding Pro2013).

Aufreinigung: The antibody was affinity-purified from rabbit antiserum by affinity chromatography using epitope-specific immunogen

Reinheit: > 95 % pure by SDS-PAGE

Anwendungsinformationen

Applikationshinweise: ELISA: 1: 10000approx. 1: 20000. WB: 1: 500approx. 1: 1000.
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Konzentration: 1.0 mg/mL

Buffer: Phosphate buffered saline (PBS), pH 7.2., 0.05 % Sodium azide

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Handhabung: Avoid repeated freezing and thawing.

Lagerung: 4 °C/-20 °C

Informationen zur Lagerung: Store undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.

Details zu Dysferlin

Target: Dysferlin (DYSF)

Andere Bezeichnung: Dysferlin (DYSF Produkte)

Synonyme: DYSF antikoerper, fb73b05 antikoerper, wu:fb73b05 antikoerper, si:rp71-50c18.1 antikoerper, DKFZp459E1226 antikoerper, 2310004N10Rik antikoerper, AI604795 antikoerper, D6Pas3 antikoerper, mFLJ00175 antikoerper, FER1L1 antikoerper, LGMD2B antikoerper, MMD1 antikoerper, dysferlin antikoerper, dysferlin, limb girdle muscular dystrophy 2B (autosomal recessive) antikoerper, myoferlin antikoerper, DYSF antikoerper, dysf antikoerper, LOC589501 antikoerper, Dysf antikoerper

Hintergrund: Dysferlin is a muscle-specific protein that is essential for normal muscle function and development. Mutations in the human dysferlin gene, DYSF, which maps to chromosome 2p13.3-p13.1, are associated with limb girdle muscular dystrophy-2B (LGMD-2B) and a related, adult-onset, distal dystrophy known as Miyoshi myopathy (MM). Dysferlin, a protein with a molecular mass of approximately 230 kDa, localizes to the muscle fiber membrane, but is absent in MM and LGMD-2B muscle. Dysferlin is detected in 5-6 week embryos, when limbs begin to form regional differentiation. Although it is not essential for initial myogenesis, dysferlin appears to be critical for sustained normal function in mature muscle. It has been suggested that the absence of dysferlin during development gives rise to the disease phenotype in adulthood. Identical mutations in the dysferlin gene can produce more than one myopathy phenotype, indicating that additional genes and/or other factors are also involved in the clinical phenotype. The DYSF gene has no homology to any other known mammalian gene, but the protein product is related to the spermatogenesis factor fer-1 of Caenorhabditis elegans. The name 'dysferlin' combines the role of the gene in producing muscular dystrophy with its homology to C. elegans.Synonyms: DYSF, Dystrophy-associated fer-1-like protein, FER1L1, Fer-1-like protein 1

Molekulargewicht: approx. 240 kDa

Gen-ID: 8291

NCBI Accession: NP_001123927

UniProt: O75923