GuineaPig Bullous Pemhpigoid ELISA Kit | BP elisa kit
Guinea Pig Bullous Pemhpigoid ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
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. Ref.7 Ref.20 Ref.23Isoform 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. Ref.7 Ref.20 Ref.23Isoform 6: required for bundling actin filaments around the nucleus
By similarity. Ref.7 Ref.20 Ref.23Isoform 7: regulates the organization and stability of the microtubule network of sensory neurons to allow axonal transport. Ref.7 Ref.20 Ref.23
Subunit structure: Homodimer. Isoform 1 interacts (via N-terminus) with PLEC (via N-terminus). Interacts with the neuronal intermediate filament protein, PRPH. Interacts with DES. Interacts with SYNE3
By similarity. Isoform 1 and isoform 6 can homodimerize (via N-terminus). Isoform 1 interacts (via N-terminus) with ACTN2. Isoform 1 interacts (via N-terminus) with PLEC (via N-terminus). Isoform 3 interacts (via N-terminus) with COL17A1 (via cytoplasmic region). Isoform 3 interacts (via N-terminus) with ITGB4 isoform beta-4a (via cytoplasmic region). Isoform 3 interacts (via N-terminus) with ERBB2IP (via C-terminus). Isoform 3 associates (via C-terminal) with KRT5-KRT14 (via rod region) intermadiate filaments of keratins. Interacts with MAPRE1; probably required for targeting to the growing microtubule plus ends. Interacts with TMIGD2. Ref.17 Ref.19 Ref.20 Ref.24 Ref.28
Subcellular location: Cytoplasm › cytoskeleton. Note: Associates with intermediate filaments, acin 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. Ref.3 Ref.7 Ref.20 Ref.21 Ref.23 Ref.24Isoform 1: Cytoplasm › cytoskeleton
By similarity. Cytoplasm › myofibril › sarcomere › Z line. Cytoplasm › myofibril › sarcomere › H zone
By similarity. Note: Localizes to microtubules and actin microfilaments throughout the cytoplasm and at focal contact attachments at the plasma membrane
By similarity. Ref.3 Ref.7 Ref.20 Ref.21 Ref.23 Ref.24Isoform 2: Cytoplasm › cytoskeleton
By similarity. Note: Colocalizes both cortical and cytoplasmic actin filaments
By similarity. Ref.3 Ref.7 Ref.20 Ref.21 Ref.23 Ref.24Isoform 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. Ref.3 Ref.7 Ref.20 Ref.21 Ref.23 Ref.24Isoform 6: Nucleus
By similarity. Nucleus envelope. Membrane; Single-pass membrane protein
By similarity. Endoplasmic reticulum membrane; Single-pass membrane protein
By similarity. Cytoplasm › cytoskeleton. Membrane; Single-pass membrane protein. 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. Ref.3 Ref.7 Ref.20 Ref.21 Ref.23 Ref.24Isoform 7: Cytoplasm › cytoskeleton. Cell projection › axon. Membrane. Note: Associates with axonal microtubules and intermediate filaments, but not with actin cytoskeleton, in sensory neurons. Ref.3 Ref.7 Ref.20 Ref.21 Ref.23 Ref.24Isoform 8: Cytoplasm › cytoskeleton
By similarity. Cytoplasm › cell cortex
By similarity. Cell membrane; Lipid-anchor
By similarity Ref.3 Ref.7 Ref.20 Ref.21 Ref.23 Ref.24.
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. Ref.3 Ref.16 Ref.24
Domain: Its association with epidermal and simple keratins is dependent on the tertiary structure induced by heterodimerization of these intermedaite filaments proteins and most likely involves recognition sites located in the rod domain of these keratins. Ref.18 Ref.29The microtubule tip localization signal (MtLS) motif; mediates interaction with MAPRE1 and targeting to the growing microtubule plus ends. Ref.18 Ref.29
Involvement in disease: Hereditary sensory and autonomic neuropathy 6 (HSAN6) [MIM:614653]: 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.1 Ref.27
Sequence similarities: Contains 1 actin-binding domain.Contains 2 CH (calponin-homology) domains.Contains 2 EF-hand domains.Contains 1 GAR domain.Contains 5 plectin repeats.Contains 1 SH3 domain.Contains 20 spectrin repeats.
Sequence caution: The sequence AAA35538.1 differs from that shown. Reason: Contaminating sequence. Sequence of unknown origin in the C-terminal part.The sequence AAA57185.1 differs from that shown. Reason: Erroneous gene model prediction. The sequence BAB70870.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.The sequence BAC04449.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.The sequence BAC04848.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.The sequence CAI14341.1 differs from that shown. Reason: Erroneous gene model prediction. The sequence CAI14989.1 differs from that shown. Reason: Erroneous gene model prediction. The sequence CAI16609.1 differs from that shown. Reason: Erroneous gene model prediction. The sequence CAI16610.1 differs from that shown. Reason: Erroneous gene model prediction. The sequence CAI20330.1 differs from that shown. Reason: Erroneous gene model prediction. The sequence CAI20332.1 differs from that shown. Reason: Erroneous gene model prediction. The sequence CAI22044.1 differs from that shown. Reason: Erroneous gene model prediction. The sequence CAI22045.1 differs from that shown. Reason: Erroneous gene model prediction. Isoform 6: The sequence AAC50244.1 differs from that shown. Reason: Frameshift at position 51.
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Product Notes
The GuineaPig BP dst (Catalog #AAA726786) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA726786 ELISA Kit recognizes GuineaPig BP. It is sometimes possible for the material contained within the vial of "Bullous Pemhpigoid, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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