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COL17A1 recombinant protein :: Collagen alpha-1(XVII) chain Recombinant Protein

Scan QR to view Datasheet Catalog #    MBS1265558
SDS-Page
Unit / Price
0.05 mg (E-Coli)  /  $180 +1 FREE 8GB USB
0.2 mg (E-Coli)  /  $490 +1 FREE 8GB USB
0.5 mg (E-Coli)  /  $715 +1 FREE 8GB USB
0.05 mg (Baculovirus)  /  $950 +1 FREE 8GB USB
1 mg (E-Coli)  /  $1,170 +1 FREE 8GB USB
0.05 mg (Mammalian-Cell)  /  $1,170 +1 FREE 8GB USB
0.5 mg (Yeast)  /  $1,170 +1 FREE 8GB USB
0.1 mg (Baculovirus)  /  $1,400 +1 FREE 8GB USB
0.1 mg (Mammalian-Cell)  /  $1,860 +2 FREE 8GB USB
1 mg (Yeast)  /  $1,860 +2 FREE 8GB USB
0.5 mg (Baculovirus)  /  $1,860 +2 FREE 8GB USB
1 mg (Baculovirus)  /  $2,760 +3 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Collagen alpha-1(XVII) chain (COL17A1), Recombinant Protein

 Also Known As   

Recombinant Human Collagen alpha-1(XVII) chain protein

 Product Synonym Names    180 kDa bullous pemphigoid antigen 2; Bullous pemphigoid antigen 2
 Product Gene Name   

COL17A1 recombinant protein

[Similar Products]
 Product Synonym Gene Name    BP180; BPAG2 [Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Sequence Positions    1253-1497, fragment
 Sequence    YLTSPDVRSF IVGPPGPPGP QGPPGDSRLL STDASHSRGS SSSSHSSSVR RGSSYSSSMS TGGGGAGSLG AGGAFGEAAG DRGPYGTDIG PGGGYGAAAE GGMYAGNGGL LGADFAGDLD YNELAVRVSE SMQRQGLLQG MAYTVQGPPG QPGPQGPPGI SKVFSAYSNV TADLMDFFQT YGAIQGPPGQ KGEMGTPGPK GDRGPAGPPG HPGPPGPRGH KGEKGDKGDQ VYAGRRRRRS IAVKP
 OMIM    113811
 3D Structure    ModBase 3D Structure for Q9UMD9
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 Host    E Coli
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 Purity/Purification    Greater than 90% as determined by SDS-PAGE.
 Tag Information    This protein contains an N-terminal tag and may also contain a C-terminal Myc-tag. N-terminal host tags may vary (His, His-SUMO, His-B2M, GST). Tag types are determined by various factors including tag-protein stability and, therefore, are subject to change; please inquire for tag information.
 Sterility    Sterile filter available upon request.
 Endotoxin    Low endotoxin available upon request.
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 Preparation and Storage    Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C.
 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 Other Notes    Small volumes of COL17A1 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Product Description specifically for COL17A1 recombinant protein

   May play a role in the integrity of hidesmosome and the attachment of basal keratinocytes to the underlying basent membrane. The 120 kDa linear IgA disease antigen is an anchoring filament component involved in dermal-epidermal cohesion. Is the target of linear IgA bullous dermatosis autoantibodies.
 Product Categories/Family for COL17A1 recombinant protein    Signal Transduction
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 SDS-Page of COL17A1 recombinant protein    COL17A1 recombinant protein SDS-Page image
(Note: Representative image, actual molecular weight may vary depending on Tag type and expression host)
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NCBI/Uniprot data below describe general gene information for COL17A1. It may not necessarily be applicable to this product.
 NCBI GI #    119829187
 NCBI GeneID    1308
 NCBI Accession #    NP_000485.3 [Other Products]
 NCBI GenBank Nucleotide #    NM_000494.3 [Other Products]
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 UniProt Primary Accession #    Q9UMD9 [Other Products]
 UniProt Secondary Accession #    Q02802; Q5JV36; Q99018; Q9NQK9; Q9UC14 [Other Products]
 UniProt Related Accession #    Q9UMD9 [Other Products]
 Molecular Weight    28.5kD
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 NCBI Official Full Name    collagen alpha-1(XVII) chain
 NCBI Official Synonym Full Names    collagen type XVII alpha 1
 NCBI Official Symbol    COL17A1 [Similar Products]
 NCBI Official Synonym Symbols   
ERED; BP180; BPA-2; BPAG2; LAD-1; BA16H23.2
[Similar Products]
 NCBI Protein Information    collagen alpha-1(XVII) chain
 UniProt Protein Name    Collagen alpha-1(XVII) chain
 UniProt Synonym Protein Names   
180 kDa bullous pemphigoid antigen 2; Bullous pemphigoid antigen 2
 UniProt Gene Name    COL17A1 [Similar Products]
 UniProt Synonym Gene Names    BP180; BPAG2; LAD-1; 97 kDa LAD antigen; 97-LAD; LABD97 [Similar Products]
 UniProt Entry Name    COHA1_HUMAN
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 NCBI Summary for COL17A1    This gene encodes the alpha chain of type XVII collagen. Unlike most collagens, collagen XVII is a transmembrane protein. Collagen XVII is a structural component of hemidesmosomes, multiprotein complexes at the dermal-epidermal basement membrane zone that mediate adhesion of keratinocytes to the underlying membrane. Mutations in this gene are associated with both generalized atrophic benign and junctional epidermolysis bullosa. Two homotrimeric forms of type XVII collagen exist. The full length form is the transmembrane protein. A soluble form, referred to as either ectodomain or LAD-1, is generated by proteolytic processing of the full length form. [provided by RefSeq, Jul 2008]
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 UniProt Comments for COL17A1    COL17A1: May play a role in the integrity of hemidesmosome and the attachment of basal keratinocytes to the underlying basement membrane. Defects in COL17A1 are a cause of generalized atrophic benign epidermolysis bullosa (GABEB). GABEB is a non- lethal, adult form of junctional epidermolysis bullosa characterized by life-long blistering of the skin, associated with hair and tooth abnormalities. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Motility/polarity/chemotaxis; Extracellular matrix

Chromosomal Location of Human Ortholog: 10q24.3

Cellular Component: basement membrane; collagen; endoplasmic reticulum lumen; extracellular region; hemidesmosome; integral to plasma membrane; intercellular junction; plasma membrane

Molecular Function: protein binding

Biological Process: cell-matrix adhesion; collagen catabolic process; epidermis development; extracellular matrix disassembly; extracellular matrix organization and biogenesis; hemidesmosome assembly; regulation of immune response

Disease: Epidermolysis Bullosa, Junctional, Non-herlitz Type; Epithelial Recurrent Erosion Dystrophy
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Product References and Citations for COL17A1 recombinant protein

   Cloning and primary structural analysis of the bullous pemphigoid autoantigen, BP180.Giudice G.J., Emery D.J., Diaz L.A.J. Invest. Dermatol. 99:243-250(1992) Cloning of the human type XVII collagen gene (COL17A1) , and detection of novel mutations in generalized atrophic benign epidermolysis bullosa.Gatalica B., Pulkkinen L., Li K., Kuokkanen K., Ryynaenen M., McGrath J.A., Uitto J.Am. J. Hum. Genet. 60:352-365(1997) The DNA sequence and comparative analysis of human chromosome 10.Deloukas P., Earthrowl M.E., Grafham D.V., Rubenfield M., French L., Steward C.A., Sims S.K., Jones M.C., Searle S., Scott C., Howe K., Hunt S.E., Andrews T.D., Gilbert J.G.R., Swarbreck D., Ashurst J.L., Taylor A., Battles J., Bird C.P., Ainscough R., Almeida J.P., Ashwell R.I.S., Ambrose K.D., Babbage A.K., Bagguley C.L., Bailey J., Banerjee R., Bates K., Beasley H., Bray-Allen S., Brown A.J., Brown J.Y., Burford D.C., Burrill W., Burton J., Cahill P., Camire D., Carter N.P., Chapman J.C., Clark S.Y., Clarke G., Clee C.M., Clegg S., Corby N., Coulson A., Dhami P., Dutta I., Dunn M., Faulkner L., Frankish A., Frankland J.A., Garner P., Garnett J., Gribble S., Griffiths C., Grocock R., Gustafson E., Hammond S., Harley J.L., Hart E., Heath P.D., Ho T.P., Hopkins B., Horne J., Howden P.J., Huckle E., Hynds C., Johnson C., Johnson D., Kana A., Kay M., Kimberley A.M., Kershaw J.K., Kokkinaki M., Laird G.K., Lawlor S., Lee H.M., Leongamornlert D.A., Laird G., Lloyd C., Lloyd D.M., Loveland J., Lovell J., McLaren S., McLay K.E., McMurray A., Mashreghi-Mohammadi M., Matthews L., Milne S., Nickerson T., Nguyen M., Overton-Larty E., Palmer S.A., Pearce A.V., Peck A.I., Pelan S., Phillimore B., Porter K., Rice C.M., Rogosin A., Ross M.T., Sarafidou T., Sehra H.K., Shownkeen R., Skuce C.D., Smith M., Standring L., Sycamore N., Tester J., Thorpe A., Torcasso W., Tracey A., Tromans A., Tsolas J., Wall M., Walsh J., Wang H., Weinstock K., West A.P., Willey D.L., Whitehead S.L., Wilming L., Wray P.W., Young L., Chen Y., Lovering R.C., Moschonas N.K., Siebert R., Fechtel K., Bentley D., Durbin R.M., Hubbard T., Doucette-Stamm L., Beck S., Smith D.R., Rogers J.Nature 429:375-381(2004) Genomic organization of collagenous domains and chromosomal assignment of human 180-kDa bullous pemphigoid antigen-2, a novel collagen of stratified squamous epithelium.Li K.H., Sawamura D., Giudice G.J., Diaz L.A., Mattei M.-G., Chu M.-L., Uitto J.J. Biol. Chem. 266:24064-24069(1991) The 97-kDa (LABD97) and 120-kDa (LAD-1) fragments of bullous pemphigoid antigen 180/type XVII collagen have different N-termini.Hirako Y., Nishizawa Y., Sitaru C., Opitz A., Marcus K., Meyer H.E., Butt E., Owaribe K., Zillikens D.J. Invest. Dermatol. 121:1554-1556(2003) The 97 kDa linear IgA bullous disease antigen is identical to a portion of the extracellular domain of the 180 kDa bullous pemphigoid antigen, BPAg2.Zone J.J., Taylor T.B., Meyer L.J., Petersen M.J.J. Invest. Dermatol. 110:207-210(1998) LAD-1, the linear IgA bullous dermatosis autoantigen, is a novel 120-kDa anchoring filament protein synthesized by epidermal cells.Marinkovich M.P., Taylor T.B., Keene D.R., Burgeson R.E., Zone J.J.J. Invest. Dermatol. 106:734-738(1996) ErratumMarinkovich M.P., Taylor T.B., Keene D.R., Burgeson R.E., Zone J.J.J. Invest. Dermatol. 106:1343-1343(1996) 97-kDa linear IgA bullous dermatosis (LAD) antigen localizes to the lamina lucida of the epidermal basement membrane.Ishiko A., Shimizu H., Masunaga T., Hashimoto T., Dmochowski M., Wojnarowska F., Bhogal B.S., Black M.M., Nishikawa T.J. Invest. Dermatol. 106:739-743(1996) Evidence that the 180-kD bullous pemphigoid antigen is a transmembrane collagen, type XVII, in a triple-helical conformation and in type II transmembrane topography.Limardo M., Arffman A., Aho S., Utto J.J. Invest. Dermatol. 106:860-860(1996) Two forms of collagen XVII in keratinocytes. A full-length transmembrane protein and a soluble ectodomain.Schaecke H., Schumann H., Hammami-Hauasli N., Raghunath M., Bruckner-Tuderman L.J. Biol. Chem. 273:25937-25943(1998) The N terminus of the transmembrane protein BP180 interacts with the N-terminal domain of BP230, thereby mediating keratin cytoskeleton anchorage to the cell surface at the site of the hemidesmosome.Hopkinson S.B., Jones J.C.Mol. Biol. Cell 11:277-286(2000) Transmembrane collagen XVII, an epithelial adhesion protein, is shed from the cell surface by ADAMs.Franzke C.-W., Tasanen K., Schaecke H., Zhou Z., Tryggvason K., Mauch C., Zigrino P., Sunnarborg S., Lee D.C., Fahrenholz F., Bruckner-Tuderman L.EMBO J. 21:5026-5035(2002) Analysis of the interactions between BP180, BP230, plectin and the integrin alpha6beta4 important for hemidesmosome assembly.Koster J., Geerts D., Favre B., Borradori L., Sonnenberg A.J. Cell Sci. 116:387-399(2003) Extracellular phosphorylation of collagen XVII by ecto-casein kinase 2 inhibits ectodomain shedding.Zimina E.P., Fritsch A., Schermer B., Bakulina A.Y., Bashkurov M., Benzing T., Bruckner-Tuderman L.J. Biol. Chem. 282:22737-22746(2007) Initial characterization of the human central proteome.Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.BMC Syst. Biol. 5:17-17(2011) Compound heterozygosity for a dominant glycine substitution and a recessive internal duplication mutation in the type XVII collagen gene results in junctional epidermolysis bullosa and abnormal dentition.McGrath J.A., Gatalica B., Li K., Dunnill M.G.S., McMillan J.R., Christiano A.M., Eady R.A.J., Uitto J.Am. J. Pathol. 148:1787-1796(1996) Three novel homozygous point mutations and a new polymorphism in the COL17A1 gene relation to biological and clinical phenotypes of junctional epidermolysis bullosa.Schumann H., Hammami-Hauasli N., Pulkkinen L., Mauviel A., Kuester W., Luethi U., Owaribe K., Uitto J., Bruckner-Tuderman L.Am. J. Hum. Genet. 60:1344-1353(1997) Collagen XVII is destabilized by a glycine substitution mutation in the cell adhesion domain Col15.Tasanen K., Eble J.A., Aumailley M., Schumann H., Baetge J., Tu H., Bruckner P., Bruckner-Tuderman L.J. Biol. Chem. 275:3093-3099(2000) Hemizygosity for a glycine substitution in collagen XVII unfolding and degradation of the ectodomain.Tasanen K., Floeth M., Schumann H., Bruckner-Tuderman L.J. Invest. Dermatol. 115:207-212(2000) A novel homozygous point mutation in the COL17A1 gene in a Chinese family with generalized atrophic benign epidermolysis bullosa.Wu Y., Li G., Zhu X.J. Dermatol. Sci. 28:181-186(2002)
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 Research Articles on COL17A1    1. Our findings implicate presumed gain-of-function COL17A1 mutations causing dominantly inherited ERED and improve understanding of the underlying pathology.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with COL17A1 recombinant proteinPathways associated with COL17A1 recombinant protein
 Reference Product  PubMed Publications
 DST recombinant protein  >17 publications with COL17A1 and DST
 LAMB3 recombinant protein  >16 publications with COL17A1 and LAMB3
 LAMC2 recombinant protein  >10 publications with COL17A1 and LAMC2
 LAMA3 recombinant protein  >8 publications with COL17A1 and LAMA3
 ITGB4 recombinant protein  >7 publications with COL17A1 and ITGB4
 ITGA6 recombinant protein  >1 publications with COL17A1 and ITGA6
 PLEC recombinant protein  >1 publications with COL17A1 and PLEC
 Products by Pathway  Pathway Diagram
 Alpha6-Beta4 Integrin Signaling Pathway antibodies  Alpha6-Beta4 Integrin Signaling Pathway Diagram
 Cell Junction Organization Pathway antibodies  Cell Junction Organization Pathway Diagram
 Cell-Cell Communication Pathway antibodies  Cell-Cell Communication Pathway Diagram
 Collagen Biosynthesis And Modifying Enzymes Pathway antibodies  Collagen Biosynthesis And Modifying Enzymes Pathway Diagram
 Collagen Degradation Pathway antibodies  Collagen Degradation Pathway Diagram
 Collagen Formation Pathway antibodies  Collagen Formation Pathway Diagram
 Degradation Of The Extracellular Matrix Pathway antibodies  Degradation Of The Extracellular Matrix Pathway Diagram
 Extracellular Matrix Organization Pathway antibodies  Extracellular Matrix Organization Pathway Diagram
 Protein Digestion And Absorption Pathway antibodies  Protein Digestion And Absorption Pathway Diagram
 Protein Digestion And Absorption Pathway antibodies  Protein Digestion And Absorption Pathway Diagram
Diseases associated with COL17A1 recombinant proteinOrgans/Tissues associated with COL17A1 recombinant protein
 Disease Name  Pubmed Publications
 Carcinoma Antibodies  >8 publications with COL17A1 and Carcinoma
 Carcinoma, Squamous Cell Antibodies  >8 publications with COL17A1 and Carcinoma, Squamous Cell
 Skin Neoplasms Antibodies  >4 publications with COL17A1 and Skin Neoplasms
 Hyperplasia Antibodies  >2 publications with COL17A1 and Hyperplasia
 Fetal Death Antibodies  >1 publications with COL17A1 and Fetal Death
 Nervous System Malformations Antibodies  >1 publications with COL17A1 and Nervous System Malformations
 Fibrosis Antibodies  >1 publications with COL17A1 and Fibrosis
 Melanoma Antibodies  >1 publications with COL17A1 and Melanoma
 Neoplasm Invasiveness Antibodies  >1 publications with COL17A1 and Neoplasm Invasiveness
 Cell Transformation, Neoplastic Antibodies  >1 publications with COL17A1 and Cell Transformation, Neoplastic
 Organ/Tissue Name  Pubmed Publications
 Skin Antibodies  >56 publications with COL17A1 and Skin
 Blood Antibodies  >5 publications with COL17A1 and Blood
 Mouth Antibodies  >4 publications with COL17A1 and Mouth
 Eye Antibodies  >4 publications with COL17A1 and Eye
 Lung Antibodies  >2 publications with COL17A1 and Lung
 Embryonic Tissue Antibodies  >1 publications with COL17A1 and Embryonic Tissue
 Kidney Antibodies  >1 publications with COL17A1 and Kidney
 Brain Antibodies  >1 publications with COL17A1 and Brain
 Intestine Antibodies  >1 publications with COL17A1 and Intestine
 Bladder Antibodies  >1 publications with COL17A1 and Bladder
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