Close
Loading...
CONTACT US
:
:
:
Call us at:
:
:
:
Fax:
Email:

DYSF

Products

Loading...

All listed services and products are For Research Use Only. Do Not use in any diagnostic or therapeutic applications.

Background

Dysferlin, encoded by the DYSF gene located on chromosome 2p13, is a 237 kDa membrane-associated protein belonging to the ferlin family—a group of proteins characterized by multiple C2 domains and a single C-terminal transmembrane domain. The DYSF gene is a 55-exon gene that is expressed in various tissues but predominantly in skeletal and cardiac muscles. The canonical human dysferlin protein contains 2,080 amino acids and features seven C2 domains (designated C2A through C2G) distributed along the peptide sequence, with approximately 13%–33% identity among the domains. These C2 domains function as calcium-sensitive modules that recruit calcium ions and traffic accessory proteins and vesicles to injured membrane sites. Dysferlin also contains two DysF domains and a single transmembrane domain near the C-terminus. As a single-pass type IV membrane protein, dysferlin is localized to the sarcolemma—the plasma membrane of muscle fibers—and to the transverse (T)-tubules, which are invaginations of the sarcolemma extending into the muscle fiber interior. Dysferlin binds to multiple interacting partners including caveolin-3, annexins, AHNAK, and calpain-3, consistent with its roles in membrane trafficking and repair.

Dysferlin plays a central role in maintaining the structural and functional integrity of striated muscle through multiple mechanisms. Upon mechanical stress or injury to the sarcolemma, calcium enters the cell through membrane lesions and triggers the mobilization of a dysferlin-associated repair complex that promotes membrane sealing. Dysferlin mediates the calcium-dependent fusion of intracellular vesicles with the plasma membrane to reseal membrane disruptions. In addition to its well-established role in membrane repair, dysferlin is involved in the development and maintenance of the T-tubule network. Recent evidence indicates that dysferlin regulates calcium homeostasis in striated muscle via multiple mechanisms, and this regulatory function becomes more important under conditions of stress. Mutations in the DYSF gene cause a group of autosomal recessive muscular dystrophies collectively known as dysferlinopathies, which include limb-girdle muscular dystrophy type 2B/R2 (LGMD2B/LGMDR2) and Miyoshi myopathy type 1 (MMD1). The loss of functional dysferlin leads to defective membrane repair, abnormal calcium handling, and progressive muscle degeneration. These properties make dysferlin an important research target for studies of membrane repair mechanisms, calcium signaling, T-tubule biology, and the molecular pathogenesis of muscular dystrophies.

Fig. 1 The Structure of DYSF. (OA Literature)Fig. 1 Domain structure of dysferlin.1

DYSF Protein Function: Roles in Membrane Repair, T-Tubule Maintenance, and Calcium Signaling

Dysferlin, encoded by the DYSF gene, functions as a calcium-sensitive membrane-associated protein with diverse roles in striated muscle physiology:

  • Calcium-Dependent Membrane Repair: Upon sarcolemmal injury, calcium enters the cell and triggers dysferlin-mediated fusion of intracellular vesicles with the plasma membrane to reseal membrane disruptions.
  • T-Tubule Development and Maintenance: Dysferlin localizes to the transverse tubules of striated muscle and is involved in maintaining T-tubule structure and function.
  • Calcium Signaling Regulation: Dysferlin regulates calcium handling in striated muscle through multiple mechanisms, a function that becomes more critical under conditions of cellular stress.
  • C2 Domain-Mediated Protein Trafficking: The seven C2 domains of dysferlin recruit calcium ions and traffic accessory proteins and vesicles to injured membrane sites, with distinct C2 domains serving specialized functions in membrane repair.
  • Protein-Protein Interactions: Dysferlin interacts with multiple partners including caveolin-3, annexins, AHNAK, and calpain-3, forming a repair complex essential for sarcolemmal integrity.

DYSF Membrane Protein Product

Creative Biolabs offers high-quality DYSF membrane protein products produced using optimized expression systems. These include full-length type IV transmembrane protein that retains the native C-terminal membrane-spanning domain, as well as isolated domain variants containing the C2 domains and DysF domains. The multiple C2 domains mediate calcium-dependent membrane binding and protein-protein interactions, making these proteins suitable for studies of membrane repair mechanisms, vesicle fusion, and protein complex assembly. All DYSF proteins undergo strict quality control to support consistent performance across applicable research platforms.

Custom DYSF Protein Product

Not finding the protein product you need? Contact us to start your one-stop custom service!

DYSF Stable Cell Line Product

Creative Biolabs provides custom-engineered DYSF stable cell lines, including overexpression and knockdown models. These cell lines are optimized for studies of membrane repair mechanisms, calcium signaling, T-tubule maintenance, and muscle cell biology. Each cell line undergoes stringent validation to ensure stable expression profiles and consistent functional performance in diverse experimental contexts.

DYSF Stable Cell Line Product

Not finding the stable cell line product you need? Contact us to start your one-stop custom service!

DYSF Recombinant Antibody Product

High-specificity recombinant antibodies targeting DYSF are developed using antibody engineering technologies for research applications involving DYSF expression and localization. These antibodies can be used in studies of DYSF distribution in skeletal and cardiac muscle cells and may also support characterization of DYSF-containing repair complexes in combination with appropriate detection reagents.

DYSF Recombinant Antibody Product

Not finding the recombinant antibody product you need? Contact us to start your one-stop custom service!

Product Features

  • Native C2 Domain Structure Preservation: Maintains the characteristic calcium-sensitive C2 domain architecture essential for membrane binding and vesicle fusion studies.
  • Membrane Repair Research Compatibility: Optimized reagent series for studies of sarcolemmal repair mechanisms, calcium-dependent vesicle fusion, and T-tubule biology.
  • High Specificity for DYSF: Minimizes non-specific cross-reactivity with other ferlin family members including myoferlin and otoferlin.
  • Comprehensive Customization Support: Facilitates end-to-end development of customized proteins, antibodies and stable cell lines to address membrane repair and muscle cell biology research demands.

Custom DYSF Research Services

Beyond catalog products, Creative Biolabs offers specialized custom services for DYSF research:

  • Custom DYSF Protein Production: Tailored expression of mutant and tagged DYSF constructs for membrane repair and calcium signaling studies.
  • Custom Antibody Development: Generation of DYSF-specific antibodies for immunostaining, flow cytometry, and protein detection applications.
  • Stable Cell Line Engineering: Construction of DYSF-modified cell models for membrane repair, calcium handling, and muscle cell biology research.
  • Functional Assay Development: Custom design of membrane repair, vesicle fusion, and calcium signaling detection workflows.

Frequently Asked Questions (FAQs)

  1. What is the primary function of DYSF?

    DYSF is a calcium-sensitive membrane-associated protein that mediates sarcolemmal membrane repair through vesicle fusion, maintains T-tubule structure, and regulates calcium signaling in striated muscle.

  2. Why is DYSF a significant research target?

    DYSF plays essential roles in membrane integrity and repair in skeletal and cardiac muscle. Mutations in DYSF cause dysferlinopathies, making it a key target for studies of membrane repair mechanisms, calcium signaling, and muscular dystrophy pathogenesis.

  3. Are Creative Biolabs' DYSF products suitable for clinical use?

    No, all DYSF products and services are strictly for research use only, not intended for clinical diagnosis or treatment.

  4. What types of DYSF products does Creative Biolabs offer?

    Offerings include full-length DYSF membrane-associated proteins, domain variants, specific detection antibodies, and custom stable cell lines for membrane repair and muscle cell biology research.

  5. How are DYSF proteins validated for activity?

    DYSF proteins are validated via calcium-dependent membrane binding assays, protein-protein interaction assays, and relevant functional characterization in applicable research platforms.

Reference
  1. Bloch, R. J., et al. "Dysferlin and the Regulation of Ca²⁺ Release in Skeletal Muscle." Cells 14.21 (2025): 1724. Under Open Access license CC BY 4.0, without modification. https://doi.org/10.3390/cells14211724
Our customer service representatives are available 24 hours a day, 7 days a week. Contact Us
© 2026 Creative Biolabs. | Contact Us
;