DST Antibody
Shipped with Ice Packs 
In Stock 
Description
What is a DST Antibody?
DST antibodies are immunoreagents designed to detect dystonin, a plakin family protein involved in anchoring intermediate filaments to cellular structures. DST has three major isoforms:
-
DST-a: Neuronal isoform, critical for maintaining cytoskeletal integrity in sensory neurons .
-
DST-b: Muscular isoform, essential for striated muscle maintenance .
-
DST-e: Epithelial isoform, anchoring keratin filaments to hemidesmosomes .
Mutations in DST are linked to hereditary sensory and autonomic neuropathy type VI (HSAN-VI) and epidermolysis bullosa simplex (EBS) . DST antibodies are used to study these pathologies and validate protein expression across tissues .
2.1. Protein Detection and Localization
-
Western Blot (WB): Detects DST isoforms (e.g., ~861 kDa full-length protein) in tissues such as brain (mouse/rat) and liver cancer samples .
-
Immunohistochemistry (IHC): Localizes DST in formalin-fixed paraffin-embedded tissues, with antigen retrieval recommended for optimal results .
-
Immunofluorescence (IF): Visualizes DST in cultured cells (e.g., SH-SY5Y neuroblastoma cells) .
2.2. Functional Studies
-
Disease Modeling: Used to investigate DST-related disorders, such as HSAN-VI and EBS, in mouse models .
-
Interaction Studies: Identified binding partners like collagen XVII, MAP1B, and erbin, elucidating DST’s role in cytoskeletal organization .
4.1. Neurological and Dermatological Disorders
-
HSAN-VI: Loss of DST-a2 disrupts neuronal autophagy, leading to sensory neuropathy .
-
EBS: DST-e deficiency causes skin blistering due to hemidesmosome instability .
4.2. Myopathy and Cardiomyopathy
-
DST-b Mutations: Cause late-onset protein aggregate myopathy and cardiomyopathy in mice, characterized by desmin aggregation and mitochondrial dysfunction .
4.3. Transplant Immunology
-
Donor-Specific Transfusion (DST): Preclinical studies show DST combined with anti-CD154 antibodies prolongs allograft survival in nonhuman primates .
Technical Considerations
-
Dilution Ranges:
-
Validation: Ensure isoform specificity (e.g., DST-a vs. DST-b) using knockout controls .
Emerging Insights and Clinical Relevance
DST antibodies have revealed novel disease mechanisms:
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
Description
This DST polyclonal antibody, CSB-PA17019A0Rb, was generated in rabbits immunized with Recombinant Human Dystonin protein (1-195AA). Dystonin (DST), a member of the plakin family of adhesion junction plaque proteins, serves as an integrator of intermediate filaments, actin, and microtubule cytoskeleton networks. It is essential for anchoring intermediate filaments to the actin cytoskeleton in neural and muscle cells or keratin-containing intermediate filaments to hemidesmosomes in epithelial cells.
This Rabbit anti-Homo sapiens (Human) DST Polyclonal antibody has been validated in ELISA, Western Blot, and Immunofluorescence applications. The non-conjugated IgG has been purified using protein G, achieving a purity level of 95%. It exhibits specific reactivity with DST proteins of human origin and can be used to detect endogenous levels of DST protein.
This Rabbit anti-Homo sapiens (Human) DST Polyclonal antibody has been validated in ELISA, Western Blot, and Immunofluorescence applications. The non-conjugated IgG has been purified using protein G, achieving a purity level of 95%. It exhibits specific reactivity with DST proteins of human origin and can be used to detect endogenous levels of DST protein.
Form
Liquid
Lead Time
Typically, we can dispatch the products within 1-3 business days following receipt of your order. Delivery times may vary depending on the purchasing method or location. Please consult your local distributors for specific delivery timelines.
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. Its role is critical 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 can 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. Additionally, it plays a structural role in the assembly of hemidesmosomes in epithelial cells, anchoring keratin-containing intermediate filaments to the inner plaque of these structures. Dystonin is required for regulating keratinocyte polarity and motility, mediating integrin ITGB4 regulation of RAC1 activity. It also facilitates the bundling of 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 study reported 2 novel heterozygous mutations in the dystonin (DST) gene from a family with hereditary sensory and autonomic neuropathy type VI. Findings using induced-pluripotent stem cells suggest that the dystonin defect might alter the proper development of peripheral nerves. PMID: 28468842
- Dystonin and bullous pemphigoid antigen 1 are encoded by the same gene but are distinct proteins with different associated diseases [review]. PMID: 26479498
- Quantitative RT-PCR analysis validated two of the six genes (LAMA3 and DST) for tumor-specific alternative splicing events. PMID: 24675808
- Four families with autosomal recessive EBS from Kuwait were identified, where the skin fragility was caused by recurrent nonsense mutations in DST-4. PMID: 25059916
- Circulating anti-BP230 autoantibodies were not correlated with the severity of genital lichen sclerosis or itching. PMID: 24676719
- BPAG1-e plays a key role in regulating keratinocyte adhesion and migration, suggesting its requirement in controlling functional switching between integrin types in epithelial cells. PMID: 24025550
- During the entry of herpes simplex virus 1, dystonin plays a specific role in the 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
- A study identified 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 component of the 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 the 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
- The 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 can also localize to the nucleus. PMID: 14576348
- Additional autoantibodies against a 230-kDa protein and 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
- The crystal structure of a stable fragment from BPAG1, residues 226-448, defined by limited proteolysis of the whole plakin domain, was determined. The plakin domain comprises 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 the expression of bullous pemphigoid antigen 1 through a 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.
Quick Inquiry
Personal Email Detected
Please use an institutional or corporate email address for inquiries. Personal email accounts ( such as Gmail, Yahoo, and Outlook) are not accepted. *
