Sign in or Register   Sign in or Register
  |  

Human Anti-DST Recombinant Antibody (H10D.Hy.8B) (CBMAB-D1809-YC)

Provided herein is a Human monoclonal antibody, which binds to Dystonin (DST). The antibody can be used for immunoassay techniques, such as IHC-Fr, ICC, IF.
See all DST antibodies

Summary

Host Animal
Human
Specificity
Human
Clone
H10D.Hy.8B
Antibody Isotype
IgG4
Application
IHC-Fr, ICC, IF

Basic Information

Immunogen
Bullous Pemphigoid Antigen was used as immunogen
Specificity
Human
Antibody Isotype
IgG4
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Dystonin
Introduction
DST belongs to 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.
Entrez Gene ID
667
UniProt ID
Q03001
Alternative Names
Dystonin; Hemidesmosomal Plaque Protein; Bullous Pemphigoid Antigen 1; Dystonia Musculorum Protein; BPAG1; BP240; BPA; DMH; DT; Bullous Pemphigoid Antigen 1, 230/240kDa; 230/240 KDa Bullous Pemphigoid Antigen; 230 KDa Bullous Pemphigoid Antigen;
Research Area
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 (By similarity).

Isoform 3:
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.

Isoform 6:
required for bundling actin filaments around the nucleus.

Isoform 7:
regulates the organization and stability of the microtubule network of sensory neurons to allow axonal transport.
Biological Process
Cell adhesion Source: UniProtKB-KW
Cell motility Source: UniProtKB
Cytoplasmic microtubule organization Source: GO_Central
Cytoskeleton organization Source: UniProtKB
Hemidesmosome assembly Source: UniProtKB
Integrin-mediated signaling pathway Source: UniProtKB
Intermediate filament cytoskeleton organization Source: UniProtKB
Maintenance of cell polarity Source: UniProtKB
Microtubule cytoskeleton organization Source: UniProtKB
Response to wounding Source: UniProtKB
Retrograde axonal transport Source: UniProtKB
Wound healing Source: GO_Central
Cellular Location
Cytoskeleton; Stress fiber; Axon. Associates with intermediate filaments, actin and microtubule cytoskeletons. Localizes to actin stress fibers and to actin-rich ruffling at the cortex of cells (By similarity). Associated at the growing distal tip of microtubules.
Isoform 1: Cytoskeleton; Z line; H zone. Localizes to microtubules and actin microfilaments throughout the cytoplasm and at focal contact attachments at the plasma membrane.
Isoform 2: Cytoskeleton. Colocalizes both cortical and cytoplasmic actin filaments.
Isoform 3: Cytoskeleton; Hemidesmosome. Localizes to actin and intermediate filaments cytoskeletons (By similarity). Colocalizes with the epidermal KRT5-KRT14 intermediate filaments network of keratins. Colocalizes with ITGB4 at the leading edge of migrating keratinocytes.
Isoform 6: Cytoskeleton; Stress fiber; Endoplasmic reticulum membrane; Nucleus; Nucleus envelope; Membrane. Localizes to actin and intermediate filaments cytoskeletons. Localizes to central actin stress fibers around the nucleus and is excluded form focal contact sites in myoblast cells. Translocates to the nucleus (By similarity). Associates with actin cytoskeleton in sensory neurons.
Isoform 7: Cytoskeleton; Axon; Membrane. Associates with axonal microtubules and intermediate filaments, but not with actin cytoskeleton, in sensory neurons.
Isoform 8: Cytoskeleton; Cell membrane; Cell cortex
Involvement in disease
Neuropathy, hereditary sensory and autonomic, 6 (HSAN6):
A form of hereditary sensory and autonomic neuropathy, a genetically and clinically heterogeneous group of disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and by sensory and/or autonomic abnormalities. HSAN6 is a severe autosomal recessive disorder characterized by neonatal hypotonia, respiratory and feeding difficulties, lack of psychomotor development, and autonomic abnormalities including labile cardiovascular function, lack of corneal reflexes leading to corneal scarring, areflexia, and absent axonal flare response after intradermal histamine injection.
Epidermolysis bullosa simplex, autosomal recessive 2 (EBSB2):
A form of epidermolysis bullosa, a dermatologic disorder characterized by localized blistering on the dorsal, lateral and plantar surfaces of the feet. EBSB2 is characterized by trauma-induced blistering mainly occurring on the feet and ankles. Ultrastructural analysis of skin biopsy shows abnormal hemidesmosomes with poorly formed inner plaques.

Motley, W. W., Züchner, S., & Scherer, S. S. (2020). Isoform-specific loss of dystonin causes hereditary motor and sensory neuropathy. Neurology Genetics, 6(5).

Yoshioka, N., Kabata, Y., Kuriyama, M., Bizen, N., Zhou, L., Tran, D. M., ... & Takebayashi, H. (2020). Diverse dystonin gene mutations cause distinct patterns of Dst isoform deficiency and phenotypic heterogeneity in D ystonia musculorum mice. Disease models & mechanisms, 13(5), dmm041608.

Lynch-Godrei, A., & Kothary, R. (2020). HSAN-VI: A spectrum disorder based on dystonin isoform expression. Neurology Genetics, 6(1).

Horie, M., Yoshioka, N., Kusumi, S., Sano, H., Kurose, M., Watanabe‐Iida, I., ... & Takebayashi, H. (2020). Disruption of dystonin in Schwann cells results in late‐onset neuropathy and sensory ataxia. Glia, 68(11), 2330-2344.

Fortugno, P., Angelucci, F., Cestra, G., Camerota, L., Ferraro, A. S., Cordisco, S., ... & Brancati, F. (2019). Recessive mutations in the neuronal isoforms of DST, encoding dystonin, lead to abnormal actin cytoskeleton organization and HSAN type VI. Human Mutation, 40(1), 106-114.

Cheng, J., Liu, H. P., Hwang, S. L., Hsu, L. F., Lin, W. Y., & Tsai, F. J. (2019). Dystonin/BPAG1 modulates diabetes and Alzheimer’s disease cross-talk: A meta-analysis. Neurological Sciences, 40(8), 1577-1582.

Jain, P. B., Guerreiro, P. S., Canato, S., & Janody, F. (2019). The spectraplakin Dystonin antagonizes YAP activity and suppresses tumourigenesis. Scientific reports, 9(1), 1-17.

Leick, K. M., Rodriguez, A. B., Melssen, M. M., Benamar, M., Lindsay, R. S., Eki, R., ... & Slingluff Jr, C. L. (2019). The barrier molecules junction plakoglobin, filaggrin, and dystonin play roles in melanoma growth and angiogenesis. Annals of surgery, 270(4), 712.

Lynch-Godrei, A., De Repentigny, Y., Gagnon, S., Trung, M. T., & Kothary, R. (2018). Dystonin-A3 upregulation is responsible for maintenance of tubulin acetylation in a less severe dystonia musculorum mouse model for hereditary sensory and autonomic neuropathy type VI. Human Molecular Genetics, 27(20), 3598-3611.

Manganelli, F., Parisi, S., Nolano, M., Tao, F., Paladino, S., Pisciotta, C., ... & Santoro, L. (2017). Novel mutations in dystonin provide clues to the pathomechanisms of HSAN-VI. Neurology, 88(22), 2132-2140.

Ask a question We look forward to hearing from you.
0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Loading...
Have you used Human Anti-DST Recombinant Antibody (H10D.Hy.8B)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon $30 eGift Card
Submit a review
Loading...
For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Documents

Contact us

  • Tel: (USA)
  • (UK)
  • Fax:
  • Email:

Submit A Review

Go to
Compare