COL17A1 Recombinant Monoclonal Antibody

Code: CSB-RA217204A0HU

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Size:

50μl100μl

US$210

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Species Reactivity: Human

Application: ELISA, WB

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Product Details
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Target Background

Product Details

Uniprot NO.

Q9UMD9

Target Names

COL17A1

Alternative Names

Collagen 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)], COL17A1, BP180 BPAG2

Species Reactivity

Human

Immunogen

A synthesized peptide derived from Human COL17A1

Conjugate

Non-conjugated

Clonality

Monoclonal

Isotype

Rabbit IgG

Clone No.

15G9

Purification Method

Affinity-chromatography

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.

Form

Liquid
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.

Scope Application

ELISA Protocol Western Blotting(WB) Protocol

Recommended Dilution

Application	Recommended Dilution
WB	1:500-1:2000

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

Datasheet & COA

Antibody FAQs

Images

Western Blot
Positive WB detected in: HEPG2 whole cell lysate
All lanes: Collagen XVII antibody at 1:1000
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 150 kDa
Observed band size: 150 kDa

Description

Collagen XVII alpha-1 chain, encoded by the COL17A1 gene, is a transmembrane protein that serves as a critical structural component of hemidesmosomes, the adhesion complexes that anchor epithelial cells to the underlying basement membrane. Also known as BP180 or bullous pemphigoid antigen 2, this protein has significant clinical relevance as an autoantigen in bullous pemphigoid and linear IgA disease, where autoantibodies target its ectodomain and cleaved fragments. Understanding COL17A1 biology is essential for researchers investigating skin integrity, autoimmune blistering disorders, and epithelial cell adhesion mechanisms.

This recombinant monoclonal antibody, clone 15G9, offers the reproducibility and consistency that demanding research applications require. Produced using recombinant technology with a defined sequence, it eliminates the lot-to-lot variability inherent in traditional hybridoma-derived antibodies, ensuring your experimental conditions remain stable across long-term studies and collaborative projects.

The antibody has been validated for Western blot applications, where it successfully detects COL17A1 in HEPG2 whole cell lysate at dilutions ranging from 1:500 to 1:2000. Validation experiments demonstrate detection of the expected 150 kDa band, consistent with the predicted molecular weight of the full-length protein. This hepatocellular carcinoma cell line provides a reliable positive control for researchers establishing detection protocols in their own systems.

Supplied in a glycerol-containing buffer optimized for long-term stability at -20°C or -80°C, this antibody is well-suited for investigators studying signal transduction pathways, cell-matrix interactions, and the molecular mechanisms underlying autoimmune skin diseases.

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Target Background

Function(From Uniprot)

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.

Gene References into Functions

Suspension survival mediated by PP2A-STAT3-Col XVII determines tumor initiation and metastasis in cancer stem cells. 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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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.

Tissue Specificity

Detected in skin. In the cornea, it is detected in the epithelial basement membrane, the epithelial cells, and at a lower level in stromal cells (at protein level). Stratified squamous epithelia. Found in hemidesmosomes. Expressed in cornea, oral mucosa,

Database Links

HGNC: 2194

UNIGENE: Hs.117938

KEGG: hsa:1308

STRING: 9606.ENSP00000340937

OMIM: 113811

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