Catalog No.
RHB35701
Species reactivity
Human, Mouse
Host species
Rabbit
Isotype
IgG
Clonality
Monoclonal
Tested applications
IF: 1:50-1:200, IHC: 1:100-1:200, WB: 1:1000-1:2000
Target
Dysferlin, Dystrophy-associated fer-1-like protein, Fer-1-like protein 1, FER1L1, DYSF
Concentration
1 mg/ml
Endotoxin level
Please contact with the lab for this information.
Purity
>95% by SDS-PAGE.
Purification
Protein A/G purified from cell culture supernatant.
Accession
O75923
Applications
IF, IHC, WB
Form
Liquid
Storage buffer
0.01M PBS, pH 7.4, 0.05% BSA, 50% Glycerol, 0.05% Sodium azide.
Stability and Storage
Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 4 ℃ for frequent use. Store at -20 ℃ for twelve months from the date of receipt.
Clone ID
R1F77
[Ultrastructural changes of skeletal muscle tissue of patients with dysferlinopathy]., PMID:39943726
Tetraspanin CD82 Associates with Trafficking Vesicle in Muscle Cells and Binds to Dysferlin and Myoferlin., PMID:37434585
Locus for severity implicates CNS resilience in progression of multiple sclerosis., PMID:37380766
In Vivo DYSF Gene Viral Delivery Provides a Histoprotective Effect in Skeletal Muscle Tissue in Dysferlin-Deficient Mice., PMID:37160600
Reactive Changes in Elements of Stromal-Vascular Differons of Dysferlin-Deficient Skeletal Muscles after Procaine Injection., PMID:33788118
Limited proteolysis as a tool to probe the tertiary conformation of dysferlin and structural consequences of patient missense variant L344P., PMID:28904177
Immunohistochemistry of sarcolemmal membrane-associated proteins in formalin-fixed and paraffin-embedded skeletal muscle tissue: a promising tool for the diagnostic evaluation of common muscular dystrophies., PMID:28219397
Dysferlin mutations and mitochondrial dysfunction., PMID:27666772
Whole Exome Sequencing Reveals DYSF, FKTN, and ISPD Mutations in Congenital Muscular Dystrophy Without Brain or Eye Involvement., PMID:25821721
Calpain cleavage within dysferlin exon 40a releases a synaptotagmin-like module for membrane repair., PMID:25143396
Expression levels of sarcolemmal membrane repair proteins following prolonged exercise training in mice., PMID:24772964
Diagnostic overview of blood-based dysferlin protein assay for dysferlinopathies., PMID:24488599
Dysferlin is a newly identified binding partner of AβPP and it co-aggregates with amyloid-β42 within sporadic inclusion-body myositis (s-IBM) muscle fibers., PMID:24091414
Full-length dysferlin expression driven by engineered human dystrophic blood derived CD133+ stem cells., PMID:24028392
[A case of dysferlinopathy asymptomatic for 10 years after an episode of transient muscle weakness]., PMID:22849992
Dystrophin and dysferlin double mutant mice: a novel model for rhabdomyosarcoma., PMID:22682622
Ex vivo stretch reveals altered mechanical properties of isolated dystrophin-deficient hearts., PMID:22427904
Immunodetection analysis of muscular dystrophies in Mexico., PMID:22334167
Unmasking potential intracellular roles for dysferlin through improved immunolabeling methods., PMID:22043020
Characterization of zebrafish dysferlin by morpholino knockdown., PMID:21893049
Myositis or dystrophy? Traps and pitfalls., PMID:21377315
Anti-TNF therapy using etanercept suppresses degenerative and inflammatory changes in skeletal muscle of older SJL/J mice., PMID:21324312
Evaluation of commercial dysferlin antibodies on canine, mouse and human skeletal muscle., PMID:20817457
Effects of rituximab in two patients with dysferlin-deficient muscular dystrophy., PMID:20618995
Novel diagnostic features of dysferlinopathies., PMID:20544924
Role of thrombospondin 1 in macrophage inflammation in dysferlin myopathy., PMID:20467328
Diverse modes of Drosophila tracheal fusion cell transcriptional regulation., PMID:20347970
Autoantibody profiles in two patients with non-autoimmune muscle disease implicate a role for gliadin autoreactivity., PMID:20110170
Differential immunohistological features of inflammatory myopathies and dysferlinopathy., PMID:19949654
Immunolabelling and flow cytometry as new tools to explore dysferlinopathies., PMID:19854055
Dysferlin deficiency treated like refractory polymyositis., PMID:19730931
The distribution and characterization of skeletal muscle lesions in dysferlin-deficient SJL and A/J mice., PMID:19615872
The susceptibility to experimental autoimmune encephalomyelitis is not related to dysferlin-deficiency., PMID:19301206
Aquaporin-4 expression is severely reduced in human sarcoglycanopathies and dysferlinopathies., PMID:18641458
Dysferlin-deficient muscular dystrophy features amyloidosis., PMID:18306167
[Dysferlin expression in limb-girdle muscular dystrophy and Miyoshi myopathy: analysis of 45 cases]., PMID:17785089
The inflammatory reaction pattern distinguishes primary dysferlinopathies from idiopathic inflammatory myopathies: an important role for the membrane attack complex., PMID:16862423
Expression of myoferlin in skeletal muscles of patients with dysferlinopathy., PMID:16707852
Analysis of histopathologic and molecular pathologic findings in Czech LGMD2A patients., PMID:16372320
Protein studies in dysferlinopathy patients using llama-derived antibody fragments selected by phage display., PMID:15827562
Immune-mediated rippling muscle disease., PMID:15668444
[A patient with distal muscular dystrophy without mutations in dysferlin gene but with abnormal dysferlin localization in muscle fibers]., PMID:15568488
In vivo and in vitro dysferlin expression in human muscle satellite cells., PMID:15535137
Clinical and pathological characteristics of four Korean patients with limb-girdle muscular dystrophy type 2B., PMID:15201514
[Dysferlin deficiency: the cause of limb-girdle muscular dystrophy 2B and Miyoshi myopathy in a Chinese pedigree]., PMID:15079794
[Two sisters with dysferlinopathy manifesting different clinical phenotypes]., PMID:12884830
Muscle pathology in dysferlin deficiency., PMID:12445162
[Recent advances in limb-girdle muscular dystrophy research]., PMID:12235836
A novel, blood-based diagnostic assay for limb girdle muscular dystrophy 2B and Miyoshi myopathy., PMID:11782994
Calpain-3 and dysferlin protein screening in patients with limb-girdle dystrophy and myopathy., PMID:11245721
For research use only. Not for human or drug use.
