Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) (DYSF) ELISA Kits

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) Antikörper (69) und und viele weitere Produktgruppen zu diesem Protein an.

list all ELISA KIts Gen GeneID UniProt
DYSF 26903 Q9ESD7
Anti-Ratte DYSF DYSF 312492  
DYSF 8291 O75923
Direkt bei antikoerper-online bestellen
  • +1 877 302 8632
  • +1 888 205 9894 (toll-free)
  • Online bestellen
  • orders@antikoerper-online.de

Top Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) ELISA Kits auf antikoerper-online.de

Showing 5 out of 12 products:

Katalog Nr. Reaktivität Sensitivität Bereich Bilder Menge Anbieter Lieferzeit Preis Details
Human 7.81 pg/mL 31.25-2000 pg/mL Typical standard curve 96 Tests Anmelden zum Anzeigen 15 bis 18 Tage
$910.56
Details
Human
  96 Tests Anmelden zum Anzeigen 2 bis 3 Tage
$380.00
Details
Maus < 46.9 pg/mL 78 pg/mL - 5000 pg/mL   96 Tests Anmelden zum Anzeigen 11 bis 18 Tage
$810.17
Details
Human < 12.5 pg/mL 31.25 pg/mL - 2000 pg/mL   96 Tests Anmelden zum Anzeigen 11 bis 18 Tage
$810.17
Details
Rind (Kuh)
  96 Tests Anmelden zum Anzeigen 15 bis 18 Tage
$1,029.60
Details

Weitere ELISA Kits für 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. Our data suggest that dysferlin modulates SR Ca(2 (zeige CA2 ELISA Kits)+) release in skeletal muscle, and that in its absence osmotic shock injury causes increased RyR1 (zeige RYR1 ELISA Kits)-mediated Ca(2 (zeige CA2 ELISA Kits)+) leak from the SR into the cytoplasm.

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

  3. 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.

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

  5. 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.

  6. 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.

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

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

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

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

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

  1. A novel duplication of 22 bases (c.897_918dup; p.Gly307Leufs5X) in the DYSF gene was identified in a family suffering from Miyoshi myopathy

  2. This review detailed the different partners and function of dysferlin and positions the sarcolemma repair in normal and pathological conditions. [Review]

  3. Immunofluorescence demonstrated that the percentage of complex I- and complex IV-deficient fibres was higher in patients with DYSF mutations than in age-matched controls. No clonally expanded mtDNA deletions were detected using long-range PCR in any of the analysed muscle fibres. Complex I and complex IV deficiency is higher in patients than age matched controls but patients do not have rearrangements of the mtDNA.

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

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

  6. Human deltoid muscle biopsies of 5 Chilean dysferlinopathy patients exhibited the presence of muscular connexins (Cx40.1, Cx43 and Cx45).

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

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

  9. 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.

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

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 ELISA Kits) translocation, membrane raft clustering and NADPH oxidase (zeige NOX1 ELISA Kits) activation in coronary arterial endothelial cells, which consequently results in endothelial dysfunction

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

Beschreibung des Gens

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
Ausgewählte Anbieter für Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) (DYSF) ELISA Kits
Haben Sie etwas anderes gesucht?