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Rabbit Anti-COL17A1 Recombinant Antibody (CBCNC-246) (V2LY-1206-LY601)


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Summary

Host Animal
Rabbit
Specificity
Human, Mouse, Rat
Clone
CBCNC-246
Antibody Isotype
IgG
Application
WB, IHC, IF, IP

Basic Information

Immunogen
Recombinant protein within Human Collagen XVII aa 1313-1420 / 1497.
Host Species
Rabbit
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
WB1:1,000-1:5,000
IHC1:50-1:200
IF(ICC)1:50

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
TBS, pH7.4, 0.05% BSA, 40% Glycerol
Preservative
0.05% sodium azide
Concentration
1 mg/ml
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Collagen Type XVII Alpha 1 Chain
Entrez Gene ID
Human1308
Mouse12821
Rat294027
UniProt ID
HumanQ9UMD9
MouseQ07563
RatD3ZSH7
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.
Biological Process
Cell-matrix adhesion Source: ProtInc
Collagen fibril organization Source: Reactome
Epidermis development Source: ProtInc
Extracellular matrix organization Source: GO_Central
Hemidesmosome assembly Source: UniProtKB
Regulation of immune response Source: Reactome
Cellular Location
Hemidesmosome; Membrane. Localized along the plasma membrane of the hemidesmosome.
120 kDa linear IgA disease antigen: Basement membrane. Exclusively localized to anchoring filaments. Localized to the epidermal side of split skin.
97 kDa linear IgA disease antigen: Basement membrane. Localized in the lamina lucida beneath the hemidesmosomes.
Involvement in disease
Generalized atrophic benign epidermolysis bullosa (GABEB):
A non-lethal, adult form of junctional epidermolysis bullosa characterized by life-long blistering of the skin, associated with hair and tooth abnormalities.
Epithelial recurrent erosion dystrophy (ERED):
A corneal dystrophy characterized by recurrent episodes of epithelial erosions from childhood, with occasional impairment of vision. Most patients have attacks of redness, photophobia, epiphora, and ocular pain. Exposure to sunlight or draught, dust and smoke and lack of sleep can precipitate attacks.
Topology
Cytoplasmic: 1-467
Helical: 468-488
Extracellular: 489-1497
PTM
The intracellular/endo domain is disulfide-linked.
Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
The ectodomain is shedded from the surface of keratinocytes resulting in a 120-kDa soluble form, also named as 120 kDa linear IgA disease antigen. The shedding is mediated by membrane-bound metalloproteases. This cleavage is inhibited by phosphorylation at Ser-544.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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