anti-Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) (DYSF) Antikörper

The protein encoded by DYSF belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. Zusätzlich bieten wir Ihnen Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) Kits (12) und und viele weitere Produktgruppen zu diesem Protein an.

Alle Antikörper anzeigen Gen GeneID UniProt
DYSF 26903 Q9ESD7
DYSF 312492  
DYSF 8291 O75923
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Top anti-Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) Antikörper auf antikoerper-online.de

Showing 10 out of 69 products:

Katalog Nr. Reaktivität Wirt Konjugat Applikation Bilder Menge Anbieter Lieferzeit Preis Details
Rind (Kuh) Kaninchen Unkonjugiert WB 100 μL Anmelden zum Anzeigen 2 bis 3 Tage
$289.00
Details
Rind (Kuh) Kaninchen Unkonjugiert WB 100 μL Anmelden zum Anzeigen 2 bis 3 Tage
$289.00
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Rind (Kuh) Kaninchen Unkonjugiert WB 100 μL Anmelden zum Anzeigen 2 bis 3 Tage
$289.00
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Human Kaninchen Unkonjugiert ELISA, WB Western blot analysis of extracts from K562 cells, using Dysferlin Antibody. The lane on the right is treated with the synthesized peptide. 100 μg Anmelden zum Anzeigen 2 bis 3 Tage
$302.50
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Rind (Kuh) Kaninchen Unkonjugiert WB 50 μg Anmelden zum Anzeigen 7 bis 9 Tage
$551.83
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Meerschweinchen Kaninchen Unkonjugiert WB 50 μg Anmelden zum Anzeigen 7 bis 9 Tage
$551.83
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Human Ziege Unkonjugiert ELISA   100 μg Anmelden zum Anzeigen 6 bis 7 Tage
$242.88
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Human Kaninchen Unkonjugiert IHC (p) 7 mL Anmelden zum Anzeigen 21 bis 31 Tage
$168.00
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Human Kaninchen Unkonjugiert IHC (p), WB 1 mL Anmelden zum Anzeigen 21 bis 31 Tage
$372.00
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Human Kaninchen Unkonjugiert EIA, IHC (p) 50 μg Anmelden zum Anzeigen 6 bis 8 Tage
$368.50
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DYSF Antikörper nach Reaktivität, Anwendung, Klonalität and Konjugat

Attribute Applikationen Wirt Klonalität Konjugat
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Am meisten referenzierte anti-Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) Antikörper

  1. Cow (Bovine) Polyclonal DYSF Primary Antibody für WB - ABIN2782235 : Leshinsky-Silver, Argov, Rozenboim, Cohen, Tzofi, Cohen, Wirguin, Dabby, Lev, Sadeh: Dysferlinopathy in the Jews of the Caucasus: a frequent mutation in the dysferlin gene. in Neuromuscular disorders : NMD 2007 (PubMed)
    Show all 2 Pubmed References

Weitere Antikörper gegen Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) Interaktionspartner

Zebrafish Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) (DYSF) Interaktionspartner

  1. zebrafish dysferlin expression is involved in stabilizing muscle structures and its downregulation causes muscle disorganization.

Mouse (Murine) Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) (DYSF) Interaktionspartner

  1. dysferlin has membrane tubulating capacity and that it shapes the T-tubule system.

  2. These results provide one mechanism by which the C57BL/6J background intensifies dysferlinopathy, giving rise to a more severe form of muscular dystrophy in the Dysf(B6) mouse model through increased membrane leak and inflammation.

  3. dysferlin-deficient cardiomyocytes showed slower Ca2 (zeige CA2 Antikörper)+ re-sequestration. Dysferlin deficiency blunted the beta-adrenergic effect on relaxation and pumping function of ex vivo working hearts.

  4. Using both naturally occurring and genetically engineered dysferlin-deficient mice, the authors demonstrated that loss of dysferlin confers increased susceptibility to coxsackievirus infection and myocardial damage.

  5. By targeting DYSF premRNA introns harbouring differentially defined 3' splice sites (3' SS), we found that target introns encoding weakly defined 3' SSs were trans-spliced successfully in vitro in human myoblasts also in vivo in skeletal muscle of mice.

  6. Dysferlin does not regulate cardiac voltage-dependent ion channels in cardiomyocytes.

  7. results show that dysferlin exerts protective effects on the fukutin (zeige FKTN Antikörper)(Hp/-) FCMD (zeige FKTN Antikörper) mouse model, and the (dysferlin(sjl/sjl): fukutin (zeige FKTN Antikörper)(Hp/-)) mice will be useful as a novel model for a recently proposed antisense oligonucleotide therapy for FCMD (zeige FKTN Antikörper)

  8. results provide the mechanism for dysferlin-mediated repair of skeletal muscle sarcolemma and identify ASM (zeige SMPD1 Antikörper) as a potential therapy for dysferlinopathy

  9. These novel observations of conspicuous intermyofibrillar lipid and progressive adipocyte replacement in dysferlin-deficient muscles.

  10. Laser-wounding induced rapid recruitment of local dysferlin-containing sarcolemma, formation of stable dysferlin accumulations surrounding lesions, endocytosis of dysferlin, and formation of large cytoplasmic vesicles from distal regions of the fiber.

Human Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) (DYSF) Interaktionspartner

  1. Data suggest that dysferlin exhibits modular architecture of 4 tertiary domains: 1) C2A, readily removed as solo domain; 2) midregion C2B-C2C-Fer-DysF, excised as intact module with several dynamic folding options; 3) C-terminal four-C2 domain module; 4) calpain-2 (zeige CAPN2 Antikörper)-cleaved mini-dysferlinC72, particularly resistant to proteolysis. Missense variant L344P in muscular dystrophy patient largely escapes proteasomal surveillance.

  2. dysferlin has membrane tubulating capacity and that it shapes the T-tubule system.

  3. Human deltoid muscle biopsies of 5 Chilean dysferlinopathy patients exhibited the presence of muscular connexins (Cx40.1, Cx43 (zeige GJA1 Antikörper) and Cx45 (zeige GJC1 Antikörper)).

  4. This review suggested that the functions of dysferlin in vesicle trafficking and membrane remodeling in skeletal muscle.

  5. DYSF expression is significantly upregulated in human masticatory mucosa during wound healing

  6. DYSF mutations in Chinese patients clustered in the N-terminal region of the gene. Exonic rearrangements were found in 23% of patients with only one pathogenic mutation identified by Sanger sequencing or NGS. The novel mutations found in this study greatly expanded the mutational spectrum of dysferlinopathy.

  7. This study showed that 4 patients with Inflammatory Myopathy associated with DYSF mutation.

  8. results support a function for dysferlin as a calcium-sensing SNARE (zeige NAPA Antikörper) effector for membrane fusion events

  9. These differences in the structural dynamics of the predicted binding site suggest that mutation R959W alters recognition dynamics of the inner DysF domain.

  10. This study demonstrated that novel mutation of DYSF in patient with Dysferlinopathy in Iran.

Rabbit Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) (DYSF) Interaktionspartner

  1. C2 domains mediate high affinity self-association of dysferlin in a parallel homodimer

Cow (Bovine) Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) (DYSF) Interaktionspartner

  1. dysferlin mediates lysosome fusion to the plasma membrane and thereby leads to ASMase (zeige SMPD1 Antikörper) translocation, membrane raft clustering and NADPH oxidase (zeige NOX1 Antikörper) activation in coronary arterial endothelial cells, which consequently results in endothelial dysfunction

Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) (DYSF) Antigen-Profil

Protein Überblick

The protein encoded by this gene belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. It is involved in muscle contraction and contains C2 domains that play a role in calcium-mediated membrane fusion events, suggesting that it may be involved in membrane regeneration and repair. In addition, the protein encoded by this gene binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in this gene have been shown to cause autosomal recessive limb girdle muscular dystrophy type 2B (LGMD2B) as well as Miyoshi myopathy. Alternative splicing results in multiple transcript variants.

Genbezeichner und Symbole assoziert mit DYSF

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

Bezeichner auf Proteinebene für DYSF

dysferlin, limb girdle muscular dystrophy 2B (autosomal recessive) , dysferlin , dysferlin-like , dysferlin variant a , dysferlin_a , dystrophy-associated fer-1-like protein , fer-1-like protein 1 , dystrophy-associated fer-1-like 1 , Dystrophy-associated fer-1-like protein , Fer-1-like protein 1

GENE ID SPEZIES
459315 Pan troglodytes
560924 Danio rerio
589501 Strongylocentrotus purpuratus
704636 Macaca mulatta
100174519 Pongo abelii
100479469 Ailuropoda melanoleuca
100488564 Xenopus (Silurana) tropicalis
100582695 Nomascus leucogenys
26903 Mus musculus
312492 Rattus norvegicus
100720513 Cavia porcellus
8291 Homo sapiens
483121 Canis lupus familiaris
100342946 Oryctolagus cuniculus
508157 Bos taurus
425353 Gallus gallus
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