Dystonin (DST) ELISA Kits

DST encodes a member of the plakin protein family of adhesion junction plaque proteins. Additionally we are shipping Dystonin Antibodies (24) and Dystonin Proteins (3) and many more products for this protein.

list all ELISA KIts Gene Name GeneID UniProt
Anti-Rat DST DST 316313  
DST 667 Q03001
DST 13518  
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Top Dystonin ELISA Kits at antibodies-online.com

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More ELISA Kits for Dystonin Interaction Partners

Human Dystonin (DST) interaction partners

  1. 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

  2. dystonin and bullous pemphigoid antigen 1 are encoded by the same gene but are different proteins with different diseases [review]

  3. Two of the six genes (LAMA3 and DST) validated by quantitative RT-PCR for tumor-specific alternative splicing events

  4. Results identify four families with autosomal recessive EBS from Kuwait in whom the skin fragility is caused by recurrent nonsense mutation in DST-4.

  5. Circulating anti-BP230 autoantibodies are not correlated with severity of genital lichen sclerosis or itching.

  6. 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.

  7. 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.

  8. Mutations of the EF-hands of BPAG1n4 abolish calcium-dependent microtubule plus end dynamics.

  9. 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

  10. 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.

  11. This is the first report of a defect in the neuronal isoform of dystonin in humans.

  12. in motile cells Col XVII recruits BPAG1e to alpha6beta4 integrin and is necessary for activation of signaling pathways, motile behavior, and lamellipodial stability.

  13. Bullous pemphigoid antigen 1 (BPAG1) was identified as a melanoma antigen recognized by its auto-antibody.

  14. 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.

  15. 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.

  16. 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.

  17. Bpag1 is not strictly a cytoplasmic/membrane protein but that it can also localize to the nucleus

  18. 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.

  19. IFN-gamma-IRF system is involved in BPAG1 gene regulation in type-1 helper T-cell inflammatory skin conditions, such as psoriasis vulgaris

  20. antibodies against BP230 can elicit the clinical and immunopathological features of Bullous pemphigoid in neonatal mice

Mouse (Murine) Dystonin (DST) interaction partners

  1. The present study identified novel mutant mice with a mild dt phenotype of sensory neurodegeneration and movement disorder.

  2. oligodendrocytes do not have an intrinsic requirement for neuronal dystonin for differentiation and myelination.

  3. Novel Dst(Gt) mice showed that a loss-of-function mutation in the actin-binding domain-containing Dystonin isoforms led to typical dystonia musculorum phenotypes

  4. In the C2.7 cell line, knockdown of BPAG1a/b had no major effect on the organization of microtubules and microfilaments, but negatively affected endocytosis and Golgi apparatus structure and decreased cell migration but not cell adhesion.

  5. Restoration of dystonin-a2 expression in the nervous system prevented the disorganization of organelle membranes and microtubule networks in a mouse model of hereditary sensory and autonomic neuropathy.

  6. This study provides insight into the mechanism of dt neuropathology and proposes a role for dystonin-a2 as a mediator of normal ER structure and function.

  7. novel functions of the dystonin-a2 isoform in mediating microtubule stability, Golgi organization, and flux through the secretory pathway.

  8. Microtubule associated protein 1B, a microtubule stabilizing protein, and clathrin heavy chain, the major component of the clathrin triskelion, were identified as interaction partners for dystonin-a

  9. dystonin does not significantly influence the structural organization of cardiac muscle fibers during early postnatal development

  10. 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.

  11. BPAG1 has three kinds of cytoskeletal binding domains and seems to play an important role in linking the different types of cytoskeletons.

  12. Transcripts for both neural isoforms of Bpag1 (a1 and a2) were detected in optic nerves and spinal cords of wild-type mice.

  13. Bpag1 may be involved in the maintenance rather than the establishment of muscle cell architecture. Cytoskeletal disruptions are seen in muscle of postnatal dystonia musculorum mice. Early phases of myogenic differentiation occur independently of Bpag1.

  14. BPAG1n4 is a novel neuronal isoform which plays an essential role in retrograde axonal transport in sensory neurons.

  15. The overall structure and subcellular localizations of Bpag1 indicate possible functions in nuclear envelope structuring, nuclear tethering, and organization of membranous structures surrounding the nucleus.

  16. These data together suggest that the survival of vagal and glossopharyngeal sensory neurons is dependent upon dystonin.

  17. Alterations likely result in reduced or absent dystonin/Bpag1 protein levels. Thus, distinct genetic defects lead to a common outcome of reduced transcript expression causing the same phenotype in multiple dt alleles.

  18. Dystonin/Bpag1 is not essential for microtubule network assembly since it is intact in short-term cultures of sensory neurons. Dystonin/Bpag1 is not an essential part of the dynein motor complex for mitochondrial transport.

  19. we characterized these N-terminal isoforms and evaluated the influence of these unique N-terminal sequences to the actin-binding properties. The N-terminal region of isoform 1 is very short and did not affect the property of the ABD that followed it.

  20. These data together suggest that dystonin is required for the innervation and development of fungiform papillae and taste buds.

Dystonin (DST) Antigen Profile

Antigen Summary

This gene encodes a member of the plakin protein family of adhesion junction plaque proteins. Multiple alternatively spliced transcript variants encoding distinct isoforms have been found for this gene, but the full-length nature of some variants has not been defined. It has been reported that some isoforms are expressed in neural and muscle tissue, anchoring neural intermediate filaments to the actin cytoskeleton, and some isoforms are expressed in epithelial tissue, anchoring keratin-containing intermediate filaments to hemidesmosomes. Consistent with the expression, mice defective for this gene show skin blistering and neurodegeneration.

Gene names and symbols associated with Dystonin (DST) ELISA Kits

  • dystonin (LOC100056885) antibody
  • dystonin (DST) antibody
  • dystonin (LOC535297) antibody
  • dystonin (Dst) antibody
  • dystonin (dst) antibody
  • dystonin L homeolog (dst.L) antibody
  • putative bullous pemphigoid antigen 1 (Smp_159110) antibody
  • dystonin (LOC100092576) antibody
  • 2310001O04Rik antibody
  • A830042E19Rik antibody
  • ah antibody
  • athetoid antibody
  • AW554249 antibody
  • BP230 antibody
  • BP240 antibody
  • BPA antibody
  • Bpag antibody
  • Bpag1 antibody
  • BPAG1-n antibody
  • CATX-15 antibody
  • CATX15 antibody
  • D6S1101 antibody
  • DKFZp469H1318 antibody
  • DMH antibody
  • dst antibody
  • dt antibody
  • fc38a05 antibody
  • HSAN6 antibody
  • Macf2 antibody
  • mKIAA0728 antibody
  • nmf203 antibody
  • nmf339 antibody
  • wu:fc38a05 antibody

Protein level used designations for Dystonin (DST) ELISA Kits

dystonin , bullous pemphigoid antigen 1 , bullous pemphigoid antigen 1, 230/240kDa , dystonia musculorum protein , hemidesmosomal plaque protein , trabeculin-beta , BPA , bullous pemphigoid antigen 1, isoforms 1/2/3/4 , bullous pemphigoid antigen 1, isoforms 6/7 , microtubule actin cross-linking factor 2 , bullous pemphigoid antigen 1, isoforms 1/2/3/4/5/8-like

100056885 Equus caballus
474948 Canis lupus familiaris
535297 Bos taurus
316313 Rattus norvegicus
667 Homo sapiens
100174353 Pongo abelii
324678 Danio rerio
421884 Gallus gallus
100302708 Saccoglossus kowalevskii
13518 Mus musculus
733202 Xenopus laevis
8354651 Schistosoma mansoni
100092576 Ornithorhynchus anatinus
100152205 Sus scrofa
100409107 Callithrix jacchus
100430705 Macaca mulatta
100542468 Meleagris gallopavo
100600859 Nomascus leucogenys
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