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DST

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. [provided by RefSeq, Mar 2010]
Full Name
Dystonin
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.

Anti-DST antibodies

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Target: DST
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: 1D2
Application*: E, IF, WB
Target: DST
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: 1B10
Application*: E, WB
Target: DST
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBYCD-430
Application*: E, IH, WB
Target: DST
Host: Human
Antibody Isotype: IgG1
Specificity: Human
Clone: H5E.Hy.4B
Application*: C, IC, IF
Target: DST
Host: Human
Antibody Isotype: IgG4
Specificity: Human
Clone: H10D.Hy.8B
Application*: C, IC, IF
Target: DST
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CAP652
Application*: Dot blot, ELISA
Target: DST
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CB314A
Application*: ELISA
Target: DST
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 2F7B3
Application*: E
For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
IFImmunofluorescence
IHImmunohistochemistry
IPImmunoprecipitation
WBWestern Blot
EELISA
MMicroarray
CIChromatin Immunoprecipitation
FFlow Cytometry
FNFunction Assay
IDImmunodiffusion
RRadioimmunoassay
TCTissue Culture
GSGel Supershift
NNeutralization
BBlocking
AActivation
IInhibition
DDepletion
ESELISpot
DBDot Blot
MCMass Cytometry/CyTOF
CTCytotoxicity
SStimulation
AGAgonist
APApoptosis
IMImmunomicroscopy
BABioassay
CSCostimulation
EMElectron Microscopy
IEImmunoelectrophoresis
PAPeptide Array
ICImmunocytochemistry
PEPeptide ELISA
MDMeDIP
SHIn situ hybridization
IAEnzyme Immunoassay
SEsandwich ELISA
PLProximity Ligation Assay
ECELISA(Cap)
EDELISA(Det)
BIBioimaging
IOImmunoassay
LFLateral Flow Immunoassay
LALuminex Assay
CImmunohistochemistry-Frozen Sections
PImmunohistologyp-Paraffin Sections
ISIntracellular Staining for Flow Cytometry
MSElectrophoretic Mobility Shift Assay
RIRNA Binding Protein Immunoprecipitation (RIP)
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