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

The protein encoded by DYSF belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. Additionally we are shipping Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) Kits (10) and and many more products for this protein.

list all antibodies Gene Name GeneID UniProt
DYSF 26903 Q9ESD7
DYSF 312492  
DYSF 8291 O75923
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Top anti-Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) Antibodies at antibodies-online.com

Showing 10 out of 62 products:

Catalog No. Reactivity Host Conjugate Application Images Quantity Supplier Delivery Price Details
Cow Rabbit Un-conjugated WB 100 μL Log in to see 2 to 3 Days
$289.00
Details
Chicken Rabbit Un-conjugated WB 100 μL Log in to see 2 to 3 Days
$289.00
Details
Human Rabbit Un-conjugated WB 100 μL Log in to see 2 to 3 Days
$289.00
Details
Human Rabbit Un-conjugated IHC (p) 7 mL Log in to see 21 to 31 Days
$161.00
Details
Human Rabbit Un-conjugated 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 Log in to see 2 to 3 Days
$302.50
Details
Human Rabbit Un-conjugated IHC (p), WB 1 mL Log in to see 21 to 31 Days
$357.00
Details
Human Rabbit Un-conjugated WB 50 μg Log in to see 8 to 10 Days
$551.83
Details
Human Rabbit Un-conjugated ELISA, WB 100 μL Log in to see 8 to 10 Days
$478.50
Details
Cow Rabbit Un-conjugated IHC, WB Immunohistochemical analysis of Dysferlin staining in human muscle formalin fixed paraffin embedded tissue section. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH 6.0). The section was then incubated with the antibody at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX. Western blot analysis of Dysferlin expression in MCF7 (A), SP2/0 (B), rat liver (C) whole cell lysates. 200 μL Log in to see 9 Days
$487.50
Details
Human Rabbit Un-conjugated EIA, IHC (p) 50 μg Log in to see 6 to 8 Days
$500.50
Details

DYSF Antibodies by Reactivity, Application, Clonality and Conjugate

Attributes Applications Host Clonality Conjugate
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Top referenced anti-Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) Antibodies

  1. Cow (Bovine) Polyclonal DYSF Primary Antibody for 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 references for ABIN2782235

  2. Chicken Polyclonal DYSF Primary Antibody for WB - ABIN2782236 : Dubowitz: Commentary from the editor. in Neuromuscular disorders : NMD 2007 (PubMed)

More Antibodies against Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) Interaction Partners

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

  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) interaction partners

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

  2. 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.

  3. 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.

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

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

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

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

  8. 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.

  9. Dysferlin, a calcium-triggered exocytotic membrane repair protein, is required for the cytoprotective effects of TRAF2 (show TRAF2 Antibodies)-mediated signaling after myocardial ischemia reperfusion injury.

  10. Alternate splicing of the dysferlin C2A domain confers Ca(2 (show CA2 Antibodies)+)-dependent and Ca(2 (show CA2 Antibodies)+)-independent binding for membrane repair.

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

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

  2. We conclude that two independent mutations in ALMS1 (show ALMS1 Antibodies) and DYSF cause CRD (show CRX Antibodies) and muscular dystrophy in the studied consanguineous Israeli Arab family.

  3. 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.

  4. Dysferlin carrier frequency and the number of affected individuals at risk for dysferlinopathy could be higher than previously estimated.

  5. Our study underlines clinical heterogeneity and a high proportion of novel mutations for dysferlin in Chinese patients affected with dysferlinopathy.

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

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

  8. Our results suggest that dysferlin protein levels of

  9. The crystal structure of the human dysferlin inner DysF domain shows that most of the pathogenic mutations are part of aromatic/arginine stacks that hold the domain in a folded conformation.

  10. The tricomplex Fam65b (show FAM65B Antibodies)-HDAC6 (show HDAC6 Antibodies)-dysferlin is transient.

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

  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) interaction partners

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

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

Protein Summary

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.

Gene names and symbols associated with DYSF

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

Protein level used designations for 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 SPECIES
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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