DST Monoclonal Antibody

Datasheet
Code CSB-MA8210361A0m
Product Type Monoclonal Antibody
Size US$350Purchase it in Cusabio online store
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Uniprot No. Q03001
Protocols ELISA Protocol
Immunogen Recombinant DST Protein
Raised in mouse
Species Reactivity Human
Specificity specific for Recombinant DST Protein denatured and native forms
Tested Applications ELISA
Relevance 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 known 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.
Form liquid
Conjugate Non-conjugated
Storage Buffer Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Purification Method >95%,protein G purifed
Isotype IgG1
Clonality monoclonal
Alias Dystonin,Bullous pemphigoid antigen,BPA
Immunogen Species Homo sapiens (Human)
Gene Names DST
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Clone No. 2F7B3
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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 (By similarity).
Involvement in disease Neuropathy, hereditary sensory and autonomic, 6 (HSAN6); Epidermolysis bullosa simplex, autosomal recessive 2 (EBSB2)
Subcellular Location Cytoplasm, cytoskeleton, Cell projection, axon, Note=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, SUBCELLULAR LOCATION: Isoform 1: Cytoplasm, cytoskeleton, Cytoplasm, myofibril, sarcomere, Z line, Cytoplasm, myofibril, sarcomere, H zone, Note=Localizes to microtubules and actin microfilaments throughout the cytoplasm and at focal contact attachments at the plasma membrane, SUBCELLULAR LOCATION: Isoform 2: Cytoplasm, cytoskeleton, Note=Colocalizes both cortical and cytoplasmic actin filaments, SUBCELLULAR LOCATION: Isoform 3: Cytoplasm, cytoskeleton, Cell junction, hemidesmosome, Note=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, SUBCELLULAR LOCATION: Isoform 6: Nucleus, Nucleus envelope, Membrane, Single-pass membrane protein, Endoplasmic reticulum membrane, Single-pass membrane protein, Cytoplasm, cytoskeleton, Note=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, SUBCELLULAR LOCATION: Isoform 7: Cytoplasm, cytoskeleton, Cell projection, axon, Membrane
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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