HPA011351
Anti-SPINK5 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
Synonym(s):
Anti-LEKTI antibody produced in rabbit, Anti-Lympho-epithelial Kazal-type-related inhibitor antibody produced in rabbit, Anti-Serine protease inhibitor Kazal-type 5 precursor antibody produced in rabbit
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All Photos(4)
About This Item
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biological source
rabbit
Quality Level
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
product line
Prestige Antibodies® Powered by Atlas Antibodies
form
buffered aqueous glycerol solution
species reactivity
human
enhanced validation
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
technique(s)
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:200-1:500
immunogen sequence
QQEERARAKAKREAAKEICSEFRDQVRNGTLICTREHNPVRGPDGKMHGNKCAMCASVFKLEEEEKKNDKEEKGKVEAEKVKREAVQELCSEYRHYVRNGRLPC
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... SPINK5(11005)
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General description
SPINK5 (serine protease inhibitor Kazal-type 5) is a secreted multi-domain serine protease inhibitor. It is expressed in hair follicles in the inner root sheets and in the epidermis in the granular layer. This protein contains 15 serine protease inhibitor domains. It is composed of 1064 amino acids, and has a signal peptide. It is synthesized as a pro-protein, which is cleaved into different isoforms. Three isoforms of this protein exist- a short isoform with only the first 13 domains, the 15 domain isoform, and a long isoform with a 30 amino acid stretch inserted between domains 13 and 14. The molecular weight of 15 domain isoform is ~120kDa. SPINK5 gene is localized to human chromosome 5q32, and is made of 33 exons.
Immunogen
Serine protease inhibitor Kazal-type 5 precursor recombinant protein epitope signature tag (PrEST)
Application
All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
Biochem/physiol Actions
SPINK5 (serine protease inhibitor Kazal-type 5) inactivates kallikrein (KLK)5, KLK7, and KLK14, which are epidermal proteases. KLKs are proteases related to desquamation, and SPINK5 is involved in the negative regulation of KLKs, as well as other proteases. Therefore, for normal skin barrier function and desquamation, a balance between KLKs and SPINK5 is essential. Mutations in this gene are associated with Comèl-Netherton syndrome, which is an autosomal recessive disorder. It is characterized by abnormalities in the hair shaft, congenital ichthyosis, and atopic diathesis. Loss of SPINK5 activity leads to increased corneodesmosomal component desmoglein-1 (DSG1) degradation, resulting in aberrant stratum corneum detachment. SPINK5 420K variant is responsible for changes in the proteolytic activation of SPINK5, which increases the likelihood of atopic dermatitis.
Features and Benefits
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
Linkage
Corresponding Antigen APREST70808
Physical form
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Legal Information
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Storage Class Code
10 - Combustible liquids
WGK
WGK 1
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Personal Protective Equipment
dust mask type N95 (US), Eyeshields, Gloves
Certificates of Analysis (COA)
Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.
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Nahoko Komatsu et al.
The Journal of investigative dermatology, 128(5), 1148-1159 (2007-11-09)
Netherton syndrome (NS) is a congenital ichthyosiform dermatosis caused by serine protease inhibitor Kazal-type 5 (SPINK5) mutations. Tissue kallikreins (KLKs) and lymphoepithelial Kazal-type-related inhibitor (LEKTI) (SPINK5 product) may contribute to the balance of serine proteases/inhibitors in skin and influence skin
Shirli Israeli et al.
Dermatology (Basel, Switzerland), 228(2), 183-188 (2014-03-01)
Comèl-Netherton syndrome is a rare congenital autosomal recessive disorder characterized by congenital ichthyosis, hair shaft abnormalities and atopic diathesis. It is caused by mutations in SPINK5, which encodes the serine protease inhibitor LEKTI. To delineate the spectrum of mutations carried
Matthieu Lacroix et al.
The Journal of investigative dermatology, 132(3 Pt 1), 575-582 (2011-11-18)
Netherton syndrome (NS) is a severe skin disease caused by loss-of-function mutations in SPINK5 (serine protease inhibitor Kazal-type 5) encoding the serine protease inhibitor LEKTI (lympho-epithelial Kazal type-related inhibitor). Here, we disclose new SPINK5 defects in 12 patients, who presented
Alessandro Tartaglia-Polcini et al.
The Journal of investigative dermatology, 126(2), 315-324 (2005-12-24)
The multidomain serine protease inhibitor lymphoepithelial Kazal-type related inhibitor (LEKTI) represents a key regulator of the proteolytic events occurring during epidermal barrier formation and hair development, as attested by the severe autosomal recessive ichthyosiform skin condition Netherton syndrome (NS) caused
Paola Fortugno et al.
Human molecular genetics, 21(19), 4187-4200 (2012-06-26)
Lymphoepithelial Kazal-type related inhibitor (LEKTI) is a multidomain serine protease inhibitor which plays a central role in skin permeability barrier and allergy. Loss-of-function mutations in the LEKTI encoding gene SPINK5 cause Netherton syndrome, a rare and severe genetic skin disease
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