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- Table of Contents
Facts about Dystonin.
Required for anchoring either intermediate filaments to the actin cytoskeleton in muscle and neural cells or keratin- containing intermediate filaments into hemidesmosomes in epithelial cells. The proteins may self-aggregate to form filaments or a two- dimensional net.
Human | |
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Gene Name: | DST |
Uniprot: | Q03001 |
Entrez: | 667 |
Belongs to: |
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No superfamily |
230 kDa bullous pemphigoid antigen; BP240; BPAFLJ30627; BPAG1FLJ32235; Bullous pemphigoid antigen 1; bullous pemphigoid antigen 1, 230/240kDa; Bullous pemphigoid antigen; CATX-15; D6S1101; DKFZp564B2416; DMHCATX15; DT; Dystonia musculorum protein; dystonin; FLJ13425; FLJ21489; FLJ46791; Hemidesmosomal plaque protein; KIAA0465; KIAA0728; KIAA1470; MACF2; trabeculin-beta
Mass (kDA):
860.662 kDA
Human | |
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Location: | 6p12.1 |
Sequence: | 6; NC_000006.12 (56457996..56954830, complement) |
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.
Cytoplasm, cytoskeleton. Cytoplasm, cytoskeleton, stress fiber. Cell projection, 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]: Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line. Cytoplasm, myofibril, sarcomere, H zone. Localizes to microtubules and actin microfilaments throughout the cytoplasm and at focal contact attachments at the plasma membrane.; [Isoform 2]: Cytoplasm, cyt
PMID: 1717441 by Sawamura D., et al. Human bullous pemphigoid antigen (BPAG1). Amino acid sequences deduced from cloned cDNAs predict biologically important peptide segments and protein domains.
PMID: 8345227 by Elgart G.W., et al. Cloning of the 5' mRNA for the 230-kD bullous pemphigoid antigen by rapid amplification of cDNA ends.