DST Antibody, FITC conjugated
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Description
Definition and Composition
DST Antibody, FITC conjugated refers to an antibody raised against the DST antigen (often part of the dystonin/epithelial protein complex) that is chemically linked to FITC. Key characteristics include:
Conjugation Methodology
FITC conjugation involves covalent attachment to lysine residues on the antibody via its isothiocyanate group . Standard protocols include:
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Antibody Preparation:
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FITC Labeling:
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Post-Conjugation Processing:
Validation and Quality Control
Key validation steps ensure functional integrity:
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Specificity:
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Brightness and Background:
Flow Cytometry
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Direct detection of cell-surface DST antigens. Example: Staining of lymphocytes with FITC-conjugated anti-DST antibodies for phenotyping .
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Double-labeling: Combined with phycoerythrin (PE)- or allophycocyanin (APC)-tagged antibodies .
Immunofluorescence Microscopy
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Subcellular localization of DST in fixed cells. Example: Tubulin network disruption studies using FITC-labeled antibodies delivered via nanoparticles .
Western Blotting
Performance Considerations
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Stability: FITC conjugates degrade under light; storage at 4°C in dark conditions is critical .
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Interference: Endogenous biotin or avidin systems require blocking to reduce background .
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Controls: Isotype-matched FITC conjugates recommended for baseline signal assessment .
Case Study: Antibody Production Without Direct Sensitization
In rat models, FITC-labeled allogeneic T cells induced anti-FITC antibody production in recipient lymph nodes, demonstrating the utility of FITC conjugates in tracking immune responses . This mirrors how FITC-conjugated DST antibodies could be used to study antigen presentation or antibody dynamics.
Product Specs
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Lead Time
Typically, we can ship the products within 1-3 business days of receiving your order. Delivery times may vary based on the purchasing method or location. For specific delivery times, please consult your local distributors.
Synonyms
DST antibody; BP230 antibody; BP240 antibody; BPAG1 antibody; DMH antibody; DT antibody; KIAA0728Dystonin antibody; 230 kDa bullous pemphigoid antigen antibody; 230/240 kDa bullous pemphigoid antigen antibody; Bullous pemphigoid antigen 1 antibody; BPA antibody; Bullous pemphigoid antigen antibody; Dystonia musculorum protein antibody; Hemidesmosomal plaque protein antibody
Target Names
DST
Uniprot No.
Target Background
Function
Dystonin is a cytoskeletal linker protein that acts as an integrator of intermediate filaments, actin, and microtubule cytoskeleton networks. It plays a crucial role in anchoring intermediate filaments to the actin cytoskeleton in neural and muscle cells or keratin-containing intermediate filaments to hemidesmosomes in epithelial cells. The proteins may self-aggregate to form filaments or a two-dimensional mesh. Dystonin regulates the organization and stability of the microtubule network of sensory neurons, enabling axonal transport. It mediates the docking of the dynein/dynactin motor complex to vesicle cargos for retrograde axonal transport through its interaction with TMEM108 and DCTN1. In addition, dystonin plays a structural role in the assembly of hemidesmosomes in epithelial cells, anchoring keratin-containing intermediate filaments to the inner plaque of hemidesmosomes. It is required for the regulation of keratinocyte polarity and motility. Dystonin mediates integrin ITGB4 regulation of RAC1 activity, is necessary for bundling actin filaments around the nucleus, and regulates the organization and stability of the microtubule network of sensory neurons to allow axonal transport.
Gene References Into Functions
- A report on 2 novel heterozygous mutations in the dystonin (DST) gene from a family with hereditary sensory and autonomic neuropathy type VI. Induced-pluripotent stem cells findings suggest that the dystonin defect might alter proper development of the peripheral nerves PMID: 28468842
- Dystonin and bullous pemphigoid antigen 1 are encoded by the same gene but are different proteins with different diseases [review] PMID: 26479498
- Two of the six genes (LAMA3 and DST) validated by quantitative RT-PCR for tumor-specific alternative splicing events PMID: 24675808
- Results identify four families with autosomal recessive EBS from Kuwait in whom the skin fragility is caused by recurrent nonsense mutation in DST-4. PMID: 25059916
- Circulating anti-BP230 autoantibodies are not correlated with severity of genital lichen sclerosis or itching. PMID: 24676719
- A key role for BPAG1-e in regulating keratinocyte adhesion and migration and suggest a requirement for this protein in controlling functional switching between integrin types in epithelial cells. PMID: 24025550
- Authors conclude that, during entry of herpes simplex virus 1, dystonin has a specific role in plus-ended transport of capsids from the centrosome to the nucleus. PMID: 23903849
- Mutations of the EF-hands of BPAG1n4 abolish calcium-dependent microtubule plus end dynamics. PMID: 22995871
- The study identifies dystonin, a cytoskeleton cross-linker involved in microtubule-based transport, as a binding partner of the HSV-1 protein pUL37, implicated in capsid transport. This study provides insight into cellular requirements for HSV-1 capsid transport and identifies dystonin as a nonmotor protein part of transport machinery PMID: 23269794
- There is a significantly enhanced ratio between the dynorphin A immunoreactive area and the whole area of the entopeduncular nucleus in genetically dystonic hamsters compared to controls. PMID: 21638337
- This is the first report of a defect in the neuronal isoform of dystonin in humans. PMID: 22522446
- In motile cells, Col XVII recruits BPAG1e to alpha6beta4 integrin and is necessary for activation of signaling pathways, motile behavior, and lamellipodial stability. PMID: 21642434
- Bullous pemphigoid antigen 1 (BPAG1) was identified as a melanoma antigen recognized by its auto-antibody. PMID: 20479946
- A homozygous nonsense mutation within the dystonin gene coding for the coiled-coil domain of the epithelial isoform of BPAG1 underlies a new subtype of autosomal recessive epidermolysis bullosa simplex. PMID: 20164846
- BPAG1-b was detectable in vitro and in vivo as a high molecular mass protein in striated and heart muscle cells, co-localizing with alpha-actinin-2 and partially with the cytolinker plectin as well as with the intermediate filament protein desmin. PMID: 19932097
- Keratinocyte responsive element 3 functions as a position-, copy number-, and orientation-dependent cis-element contributing to tissue-specific regulation of the 230-kDa bullous pemphigoid antigen gene. PMID: 12542537
- BPAG1 is not strictly a cytoplasmic/membrane protein but that it can also localize to the nucleus PMID: 14576348
- Additional autoantibodies against a 230-kDa protein and against a 190-kDa protein comigrating with bullous pemphigoid antigen 1 (BP230) and periplakin, respectively, were present in all the patients' sera. PMID: 14705806
- The IFN-gamma-IRF system is involved in BPAG1 gene regulation in type-1 helper T-cell inflammatory skin conditions, such as psoriasis vulgaris PMID: 15560761
- Antibodies against BP230 can elicit the clinical and immunopathological features of Bullous pemphigoid in neonatal mice PMID: 15725571
- Findings demonstrated that disruption of the IFN-stimulated responsive element sequences, but not the IFNgamma activation site, markedly suppressed the BPAG1 basal promoter activity and resulted in attenuated IFNgamma response in keratinocytes PMID: 16512878
- We report the crystal structure of a stable fragment from BPAG1, residues 226-448, defined by limited proteolysis of the whole plakin domain. The plakin domains has two pairs of spectrin repeats interrupted by a putative Src-Homology 3 (SH3) domain. PMID: 17161423
- IgE autoantibodies to BP180 and BP230 are detected at high frequencies in bullous pemphigoid. PMID: 17920818
- Vitamin D(3) inhibits expression of bullous pemphigoid antigen 1 through post-transcriptional mechanism without new protein synthesis. PMID: 18207369
- BPAG1e is required for efficient regulation of keratinocyte polarity and migration by determining the activation of Rac1. PMID: 19403692
Database Links
Involvement In Disease
Neuropathy, hereditary sensory and autonomic, 6 (HSAN6); Epidermolysis bullosa simplex, autosomal recessive 2 (EBSB2)
Subcellular Location
Cytoplasm, cytoskeleton. Cytoplasm, cytoskeleton, stress fiber. Cell projection, axon.; [Isoform 1]: Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line. Cytoplasm, myofibril, sarcomere, H zone.; [Isoform 2]: Cytoplasm, cytoskeleton.; [Isoform 3]: Cytoplasm, cytoskeleton. Cell junction, hemidesmosome.; [Isoform 6]: Nucleus. Nucleus envelope. Membrane; Single-pass membrane protein. Endoplasmic reticulum membrane; Single-pass membrane protein. Cytoplasm, cytoskeleton. Cytoplasm, cytoskeleton, stress fiber.; [Isoform 7]: Cytoplasm, cytoskeleton. Cell projection, axon. Membrane. Note=Associates with axonal microtubules and intermediate filaments, but not with actin cytoskeleton, in sensory neurons.; [Isoform 8]: Cytoplasm, cytoskeleton. Cytoplasm, cell cortex. Cell membrane; Lipid-anchor.
Tissue Specificity
Isoform 1 is expressed in myoblasts (at protein level). Isoform 3 is expressed in the skin. Isoform 6 is expressed in the brain. Highly expressed in skeletal muscle and cultured keratinocytes.
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