Alternative Name(s):

230KDA bullous pemphigoid antigen230/240KDA bullous pemphigoid antigen;Bullous pemphigoid antigen 1 ;BPA ;Bullous pemphigoid antigenDystonia musculorum proteinHemidesmosomal plaque protein

Species: (Organism)

Homo sapiens (Human)

Gene Names:

DST

Tag info:

N-terminal 6xHis-tagged

Target Protein AA Sequence:

MHSSSYSYRSSDSVFSNTTSTRTSLDSNENLLLVHCGPTLINSCISFGSESFDGHRLEMLQQIANRVQRDSVICEDKLILAGNALQSDSKRLESGVQFQNEAEIAGYILECENLLRQHVIDVQILIDGKYYQADQLVQRVAKLRDEIMALRNECSSVYSKGRILTTEQTKLMISGITQSLNSGFAQTLHPSLTSG

Expression Region:

1-195aa

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.

Protein Length:

Partial of Isoform 3

Pathway:

Mol. Weight:

23.7 kDa

Purity:

Greater than 90% as determined by SDS-PAGE.

Form:

Liquid or Lyophilized powder

Buffer:

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.

Research Areas:

Signal Transduction

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)

Relevance:

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 hidesmosomes in epithelial cells. The proteins may self-aggregate to form filaments or a two-dimensional mesh.Isoform 3: plays a structural role in the assbly of hidesmosomes of epithelial cells; anchors keratin-containing intermediate filaments to the inner plaque of hidesmosomes. 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.

Reconstitution:

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Protein Families:

Reference:

Complete sequencing and characterization of 21,243 full-length human cDNAs.Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.Nat. Genet. 36:40-45(2004)