Recombinant Human Collagen alpha-1 (COL17A1) Protein (His)

Name: Recombinant Human Collagen alpha-1 (COL17A1) Protein (His)
Brand: Beta LifeScience
SKU: BLC-04961P
Availability: InStock

Greater than 90% as determined by SDS-PAGE.

Collections: All products, High-quality recombinant proteins, Other recombinant proteins

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Product Overview

Description	Recombinant Human Collagen alpha-1 (COL17A1) Protein (His) is produced by our E.coli expression system. This is a protein fragment.
Purity	Greater than 90% as determined by SDS-PAGE.
Uniprotkb	Q9UMD9
Target Symbol	COL17A1
Synonyms	COL17A1; BP180; BPAG2Collagen alpha-1(XVII) chain; 180 kDa bullous pemphigoid antigen 2; Bullous pemphigoid antigen 2) [Cleaved into: 120 kDa linear IgA disease antigen; 120 kDa linear IgA dermatosis antigen; Linear IgA disease antigen 1; LAD-1); 97 kDa linear IgA disease antigen; 97 kDa linear IgA bullous dermatosis antigen; 97 kDa LAD antigen; 97-LAD; Linear IgA bullous disease antigen of 97 kDa; LABD97)]
Species	Homo sapiens (Human)
Expression System	E.coli
Tag	N-6His
Target Protein Sequence	YLTSPDVRSFIVGPPGPPGPQGPPGDSRLLSTDASHSRGSSSSSHSSSVRRGSSYSSSMSTGGGGAGSLGAGGAFGEAAGDRGPYGTDIGPGGGYGAAAEGGMYAGNGGLLGADFAGDLDYNELAVRVSESMQRQGLLQGMAYTVQGPPGQPGPQGPPGISKVFSAYSNVTADLMDFFQTYGAIQGPPGQKGEMGTPGPKGDRGPAGPPGHPGPPGPRGHKGEKGDKGDQVYAGRRRRRSIAVKP
Expression Range	1253-1497aa
Protein Length	Partial
Mol. Weight	28.4kDa
Research Area	Signal Transduction
Form	Liquid or Lyophilized powder
Buffer	Liquid form: default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.
Reconstitution	Briefly centrifuged the vial prior to opening to bring the contents to the bottom. Reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL. It is recommended to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. The default final concentration of glycerol is 50%.
Storage	1. Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. 2. Avoid repeated freeze-thaw cycles. 3. Store working aliquots at 4°C for up to one week. 4. In general, protein in liquid form is stable for up to 6 months at -20°C/-80°C. Protein in lyophilized powder form is stable for up to 12 months at -20°C/-80°C.
Notes	Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.

Target Details

Target Function	May play a role in the integrity of hemidesmosome and the attachment of basal keratinocytes to the underlying basement 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.
Subcellular Location	Cell junction, hemidesmosome. Membrane; Single-pass type II membrane protein. Note=Localized along the plasma membrane of the hemidesmosome.; [120 kDa linear IgA disease antigen]: Secreted, extracellular space, extracellular matrix, basement membrane. Note=Exclusively localized to anchoring filaments. Localized to the epidermal side of split skin.; [97 kDa linear IgA disease antigen]: Secreted, extracellular space, extracellular matrix, basement membrane. Note=Localized in the lamina lucida beneath the hemidesmosomes.
Database References	HGNC: 2194 OMIM: 113811 KEGG: hsa:1308 STRING: 9606.ENSP00000340937 UniGene: PMID: 27306323 Bullous pemphigoid patients with the HLA-DQB1*03:01 allele show an increased T-cell avidity to several epitopes of BP180, particularly the BP180-NC16a domain. (Review) PMID: 28101965 C-terminal processing induces dynamic structural changes and neoepitopes for linear IgA dermatosis autoantibodies on COL17 PMID: 28842325 As a consequence of altered maturation and decreased stability of collagen XVII trimers, reduced collagen XVII is incorporated into the cell membrane, resulting in compromised dermal-epidermal adhesion. Taken together, using this genetic model, we provide the first proof that alteration of the coiled-coil structure destabilizes oligomerization and impairs physiological shedding of collagen XVII in vivo. PMID: 28365758 Pathogenicity of autoantibodies targeting COL17 is epitope dependent. PMID: 26827765 The corneal dystrophy mapped to chromosome 10q23-q24 is associated with the c.3156C>T variant in COL17A1. PMID: 27309958 Data suggest that type XVII collagen (COL17) internalization/macropinocytosis by keratinocytes induced by IgG autoantibodies from patients with bullous pemphigoid (BP) is mediated by PKC (protein kinase C) calcium signaling pathway; BP IgG induced overabundant phosphorylation of COL17. PMID: 27775687 we have produced five new versatile monoclonal antibodies against the Col 17 endodomain that are superior to ectodomain antibodies in diagnosing and differentiating between JEB-gen intermed and JEB-loc/carriers PMID: 26334130 Results show that aberrant epigenetic control is a key driver of COL17A1 gene misexpression and tumor cell invasion in a variety of epithelial neoplasm. These findings have significant clinical implications, suggesting that the COL17A1 promoter methylation status can be used to predict patient outcome. PMID: 27891193 Case Report: IgA autoantibodies targeting the NC16A domain rather than the shed ectodomains of COL17 resulted in linear IgA bullous dermatosis in a pregnant woman. PMID: 27786348 ELISA for IgE anti-NC16a is a helpful parameter in the modification of current treatment and the assessment of risk of relapse in bullous pemphigoid. PMID: 25791894 R1303Q in COL17 hampers C-terminal cleavage of COL17. Increase in remnants of non-cleaved COL17 ectodomain in extracellular matrix (ECM) induces aberrant laminin 332 deposition in ECM, which may be associated with disorganized basement membrane formation. PMID: 26604146 The COL17A1 c.3156C-->T variant is the likely causative mutation in our recurrent corneal erosion families, and its presence in 4 independent families suggests that it is prevalent in epithelial recurrent erosion dystrophy. PMID: 26786512 Elevated serum levels of BP180 antibodies in the first trimester of pregnancy precede gestational pemphigoid and remain elevated for a long time after remission of the disease. PMID: 25758329 Letter/Case Report: IgE BP180 antibodies contribute to the occurrence of urticarial erythema in bullous pemphigoid patients. PMID: 25771164 IgE anti-BP180 autoantibody level is increased in some Chinese patients with bullous pemphigoid. PMID: 25797173 The aging process can be recapitulated by Col17a1 deficiency and prevented by the forced maintenance of COL17A1 in hair follicle stem cell (HFSCs), demonstrating that COL17A1 in HFSCs orchestrates the stem cell-centric aging program of the epithelial mini-organ. PMID: 26912707 Our findings implicate presumed gain-of-function COL17A1 mutations causing dominantly inherited ERED and improve understanding of the underlying pathology. PMID: 25676728 Case Reports: two Japanese patients with bullous pemphigoid with only BP230 autoantibodies detected by ELISA. PMID: 24676568 Circulating anti-BP180 autoantibodies are not correlated with severity of genital lichen sclerosis or itching. PMID: 24676719 study reports for the first time the expression of collagen XVII in colon epithelium and the association of increased collagen XVII immunoexpression with poor outcome in colorectal carcinoma. PMID: 25623077 Variants of the PTCH1 and COL17A1 genes may contribute to the development of Ossification of the posterior longitudinal ligament. PMID: 24668667 Lack of C17 led to decreased melanin intensity and melanocyte density in the epidermis when compared with the revertant patches. In human skin, melanocyte supply to the epidermis depends on C17 expression in keratinocytes. PMID: 24330315 Missense mutation R1303Q in COL17a1 causes junctional epidermolysis bullosa phenotype similar to Kindler syndrome. PMID: 24005051 Titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (r = 0.557, P value < 0.0001) and ABSIS (r = 0.570, P value < 0.0001) values. PMID: 23227089 The results of this study suggest that BP180 internalization induced by bullous pemphigoid IgG plays an important role in the initiation of disease pathogenesis. PMID: 23337823 found the full-length collagen XVII protein in proliferating tissue melanocytes, basal keratinocytes and squamous cell carcinoma whereas resting melanocytes were negative PMID: 22688676 Collagen XVII (BP180) modulates keratinocyte expression of the proinflammatory chemokine, IL-8. PMID: 22775995 Collagen XVII has a function in the attachment of podocyte foot processes to the glomerular basement membrane. PMID: 22457199 migrating keratinocytes shed the Ecto-ColXVII, and this dynamically binds via its C-terminal domain to distinct partners in the ECM PMID: 21801871 presence of minor amounts of collagen XVII protein in JEB skin is associated with mild phenotypic manifestations. PMID: 21357940 Cell surface COL17 can interact with laminin 332 and, together, participate in the adherence of a cell to the extracellular matrix. PMID: 21034821 Alterations in type I hemidesmosome components (BP180 and BP230) are suggestive of epigenetic control in the salivary glands of patients with Sjogren's syndrome. PMID: 21305504 small proportion of pregnant women produce protein-specific IgE autoantibodies PMID: 20471095 anti-hBPAG2 IgG was initially directed against extracelllar domain epitopes;humoral responses subsequently targeted additional extra and intracellular domain BPAG2 epitopes PMID: 19812601 Depletion of CD4-positive T cells as well as CD45R-positive B cells in an immunodeficient transgenic mouse model of bullous pemphigoid inhibits production of anti-human COL17 IgG antibodies in the recipients, resulting in no apparent clinical phenotype. PMID: 20089696 The role of collagen XVII in both autoimmune and genetic blistering disorders demonstrates its relevance to dermal-epidermal adhesion PMID: 19945617 We describe a Chinese family with generalized atrophic benign epidermolysis bullosa (GABEB). serine to cysteine at position 265. novel polymorphic substitution of C-to-G at nucleotide position 798 in exon 10 of the COL17A1 gene, an I233M change in BPAG2 PMID: 11912005 this protein, an epithelial adhesion protein, is shed from the cell surface by ADAMs PMID: 12356719 Mutations in the coding sequence of the human collagen XVII (COL17A1) gene are not the cause of Thiel-Behnke Corneal Dystrophy. PMID: 14562173 truncation of the intracellular domain of BP180 impairs the organization of hemidesmosomes, affecting both the mechanical stability of basal keratinocytes and dermoepidermal cohesion. PMID: 14962091 bullous pemphigoid sera reacted with at least an additional antigenic site other than the NC16A, within the extracellular (37%) and intracellular (28%) domains of BP180. PMID: 14962097 genetic variation in COL17A1 shows no association with susceptibility to bullous pemphigoid. PMID: 14987253 Dimished, but correctly localised expression of BP180 in epidermolysis bullosa; COL15 mutated BP180 is still partly functional. PMID: 15009107 the conformation of the NC16A domain and steric availability of the cleavage site influence shedding and is important for folding of collagen XVII PMID: 15047704 C-terminus of collagen XVII binds to laminin 5, revealing the role of collagen XVII in the regulation of keratinocyte migration. PMID: 15161638 Data show that the expression of laminin gamma2 chain and collagen type XVII is altered in endometrial adenocarcinomas. PMID: 15609083 expression of BP180 is related to clinical severity of bullous pemphigoid PMID: 15734283 Epitooe mapping of anti-BP180 immunoglobulin E autoantibodies in bullous pemphigoid. PMID: 16117787 Deletions in recombinant proteins affect thermal stability. PMID: 16354180

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Proteins are sensitive to heat, and freeze-drying can preserve the activity of the majority of proteins. It improves protein stability, extends storage time, and reduces shipping costs. However, freeze-drying can also lead to the loss of the active portion of the protein and cause aggregation and denaturation issues. Nonetheless, these adverse effects can be minimized by incorporating protective agents such as stabilizers, additives, and excipients, and by carefully controlling various lyophilization conditions.

Commonly used protectant include saccharides, polyols, polymers, surfactants, some proteins and amino acids etc. We usually add 8% (mass ratio by volume) of trehalose and mannitol as lyoprotectant. Trehalose can significantly prevent the alter of the protein secondary structure, the extension and aggregation of proteins during freeze-drying process; mannitol is also a universal applied protectant and fillers, which can reduce the aggregation of certain proteins after lyophilization.

Our protein products do not contain carrier protein or other additives (such as bovine serum albumin (BSA), human serum albumin (HSA) and sucrose, etc., and when lyophilized with the solution with the lowest salt content, they often cannot form A white grid structure, but a small amount of protein is deposited in the tube during the freeze-drying process, forming a thin or invisible transparent protein layer.

Reminder: Before opening the tube cap, we recommend that you quickly centrifuge for 20-30 seconds in a small centrifuge, so that the protein attached to the tube cap or the tube wall can be aggregated at the bottom of the tube. Our quality control procedures ensure that each tube contains the correct amount of protein, and although sometimes you can't see the protein powder, the amount of protein in the tube is still very precise.

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