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 (12) 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 69 products:

Catalog No. Reactivity Host Conjugate Application Images Quantity Supplier Delivery Price Details
Cow Rabbit Un-conjugated WB WB Suggested Anti-DYSF Antibody Titration:  0.2-1 ug/ml  ELISA Titer:  1:12500  Positive Control:  Human Muscle 100 μL Log in to see 2 to 3 Days
$289.00
Details
Cow Rabbit Un-conjugated WB WB Suggested Anti-DYSF Antibody Titration:  0.2-1 ug/ml  ELISA Titer:  1:312500  Positive Control:  Human Muscle 100 μL Log in to see 2 to 3 Days
$289.00
Details
Cow Rabbit Un-conjugated WB WB Suggested Anti-DYSF Antibody Titration:  0.2-1 ug/ml  ELISA Titer:  1:1562500  Positive Control:  Human Muscle 100 μL Log in to see 2 to 3 Days
$289.00
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 13 to 14 Days
$487.50
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, IHC (p) Immunohistochemistry: Dysferlin Antibody [NBP1-84695] - Staining of human kidney shows strong positivity in the glomeruli and moderate membranous staining in tubular cells. Immunohistochemistry-Paraffin: Dysferlin Antibody  - Staining of human skeletal muscle shows moderate cytoplasmic positivity in myocytes. 0.1 mL Log in to see 10 to 13 Days
$491.63
Details
Human Rabbit Un-conjugated ICC, IF, IHC, IHC (p), WB Immunocytochemistry/Immunofluorescence: Dysferlin Antibody (JF05-54)  - Staining Dysferlin in SW480 cells (red). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS. Immunohistochemistry-Paraffin: Dysferlin Antibody (JF05-54)  - Analysis of paraffin-embedded human spleen tissue using anti-Dysferlin antibody. Counter stained with hematoxylin. 100 μL Log in to see 34 Days
$373.75
Details
Human Rabbit Un-conjugated IHC, IHC (p), WB Anti-DYSF / Dysferlin antibody IHC of human heart. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody ABIN1804068 dilution 1:50. Anti-DYSF / Dysferlin antibody IHC of human heart. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody dilution 1:50. 250 μL Log in to see 11 to 14 Days
$484.00
Details
Guinea Pig Rabbit Un-conjugated WB 50 μg Log in to see 7 to 9 Days
$551.83
Details
Cow Rabbit Un-conjugated WB 50 μg Log in to see 7 to 9 Days
$551.83
Details

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 Pubmed References

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. Our data suggest that dysferlin modulates SR Ca(2 (show CA2 Antibodies)+) release in skeletal muscle, and that in its absence osmotic shock injury causes increased RyR1 (show RYR1 Antibodies)-mediated Ca(2 (show CA2 Antibodies)+) 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 (show CA2 Antibodies)+ 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 (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)

  9. results provide the mechanism for dysferlin-mediated repair of skeletal muscle sarcolemma and identify ASM (show SMPD1 Antibodies) 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) interaction partners

  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 (show FER Antibodies)-DysF, excised as intact module with several dynamic folding options; 3) C-terminal four-C2 domain module; 4) calpain-2 (show CAPN2 Antibodies)-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 (show GJD4 Antibodies), Cx43 (show GJA1 Antibodies) and Cx45 (show GJC1 Antibodies)).

  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) 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 (DYSF) antibody
  • dysferlin, limb girdle muscular dystrophy 2B (autosomal recessive) (dysf) antibody
  • myoferlin (LOC589501) 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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