Rabbit Anti-Collagen XVII/BP36/FITC Conjugated antibody

Home > Product > Antibody > Rabbit Anti-Collagen XVII/BP36/FITC Conjugated antibody

Bullous Pemphigoid 180; 180 kDa bullous pemphigoid antigen 2; Alpha 1 type XVII collagen; BA16H23.2; BP 180; BP180; BPAG 2; BPAG2; Bullous pemphigoid antigen 2; COL17A1; Collagen 17; Collagen alpha 1 XVII chain; Collagen alpha 1(XVII) chain; Collagen alph

Cat:

SL13964R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Dog,Pig,Cow,Horse,Rabbit,Sheep,)

Immunogen：

KLH conjugated synthetic peptide derived from human Collagen XVII

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

150kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
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]

Function:
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 the gene coding for collagen XVII 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. Two transcript variants, one resulting from alternative splicing in the 3' UTR, have been identified for this gene.

Subunit:
Homotrimers of alpha 1(XVII)chains. Interacts (via cytoplasmic region) with ITGB4 (via cytoplasmic region). Interacts (via cytoplasmic region) with DST isoform 3 (via N-terminus). Interacts (via N-terminus) with PLEC. Interacts (via cytoplasmic region) with DSP.

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 and 97 kDa linear IgA disease antigen: Secreted, extracellular space, extracellular matrix, basement membrane.

Post-translational modifications:
The intracellular/endo domain is disulfide-linked.

DISEASE:
Generalized atrophic benign epidermolysis bullosa (GABEB) [MIM:226650]: A non-lethal, adult form of junctional epidermolysis bullosa characterized by life-long blistering of the skin, associated with hair and tooth abnormalities. Note=The disease is caused by mutations affecting the gene represented in this entry.

Database links:

Entrez Gene: 1308 Human

Omim: 22811 Human

SwissProt: Q9UMD9 Human

Unigene: 117938 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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