Product Details

Uniprot No.

Target Names

DST

Alternative Names

Dystonin (230 kDa bullous pemphigoid antigen) (230/240 kDa bullous pemphigoid antigen) (Bullous pemphigoid antigen 1) (BPA) (Bullous pemphigoid antigen) (Dystonia musculorum protein) (Hemidesmosomal plaque protein), DST, BP230 BP240 BPAG1 DMH DT KIAA0728

Species Reactivity

Human

Immunogen

Recombinant Human DST protein

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Clonality

Monoclonal

Isotype

hIgG1

Clone No.

1E2

Purification Method

Affinity-chromatography

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4

Form

Liquid

Tested Applications

ELISA, IF, FC

Recommended Dilution

Application	Recommended Dilution
IF	1:50-1:200
FC	1:50-1:200

Protocols

ELISA Protocol
Immunofluorescence (IF) Protocol
Flow Cytometry (FC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Description

Dystonin, also known as bullous pemphigoid antigen 1 (BPAG1) or BP230, is a large cytoskeletal linker protein essential for maintaining cellular integrity across multiple tissue types. This plakin family member plays critical roles in anchoring intermediate filaments to hemidesmosomes in epithelial cells and contributes to neuronal cytoskeletal organization. Mutations in the DST gene are associated with hereditary sensory autonomic neuropathy and epidermolysis bullosa simplex, making it a significant target for researchers investigating cytoskeletal dynamics, cell adhesion disorders, and neurodegenerative conditions.

This recombinant monoclonal antibody (clone 1E2) offers the reproducibility and consistency that demanding research applications require. Produced using recombinant technology with a defined sequence, it eliminates the lot-to-lot variability inherent in traditional hybridoma-derived antibodies, ensuring your experimental results remain comparable across studies and over time. The human IgG1 isotype format and affinity-chromatography purification deliver a reliable reagent for quantitative analyses.

Validated for immunofluorescence and flow cytometry applications, this antibody has demonstrated clear performance in human cell models. Immunofluorescence studies in HeLa and SH-SY5Y cells show specific staining patterns when used at dilutions of 1:30 to 1:200, providing flexibility to optimize signal intensity for your experimental conditions. Flow cytometry validation in HeLa cells confirms robust detection with recommended dilutions of 1:50 to 1:200, with clear separation from isotype control signals.

Whether investigating hemidesmosomal assembly in epithelial biology, exploring cytoskeletal organization in neuronal models, or studying autoimmune blistering diseases, this DST antibody provides a dependable tool for advancing your research into this multifunctional cytoskeletal protein.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Function

Cytoskeletal linker protein. Acts as an integrator of intermediate filaments, actin and microtubule cytoskeleton networks. Required for anchoring either 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. Regulates the organization and stability of the microtubule network of sensory neurons to allow axonal transport. Mediates docking of the dynein/dynactin motor complex to vesicle cargos for retrograde axonal transport through its interaction with TMEM108 and DCTN1.; plays a structural role in the assembly of hemidesmosomes of epithelial cells; anchors keratin-containing intermediate filaments to the inner plaque of hemidesmosomes. Required for the regulation of keratinocyte polarity and motility; mediates integrin ITGB4 regulation of RAC1 activity.; required for bundling actin filaments around the nucleus.; 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
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; 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
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

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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.

Database Links

HGNC: 1090

OMIM: 113810

KEGG: hsa:667

STRING: 9606.ENSP00000244364

UniGene: Hs.604915

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Code	CSB-RA821036MA1HU
Size	US$210
	Order now
Image	Immunofluorescence staining of Hela cell with CSB-RA821036MA1HU at 1:30 ,counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Human IgG(H+L). Immunofluorescence staining of SH-SY5Y cell with CSB-RA821036MA1HU at 1:30 ,counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Human IgG(H+L). Overlay Peak curve showing Hela cells stained with CSB-RA821036MA1HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for 10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/110⁶cells) for 45min at 4℃. The secondary antibody used was Fluorescein (FITC) AffiniPure Goat Anti-Human IgG, Fcγ fragment specific at 1:200 dilution for 35 min at 4℃.Control antibody (green line) was human IgG1 (1ug/110⁶cells) used under the same conditions. Acquisition of >10,000 events was performed.
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DST Recombinant Monoclonal Antibody

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Target Background