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Code
OSA00162W
$240USD
ID Tag
Rb1727-020111-WS
Immunogen
A synthetic peptide from the n-terminal region of human ATP2C1 conjugated to an immunogenic carrier protein was used as the antigen.
Accession
Also known
Calcium-transporting ATPase type 2C member 1, ATPase 2C1, ATP-dependent Ca(2+) pump PMR1
Target
FUNCTION: This magnesium-dependent enzyme catalyzes the hydrolysis of ATP coupled with the transport of the calcium.
Defects in ATP2C1 are the cause of Hailey-Hailey disease (HHD); also known as benign familial pemphigus. HHD is an autosomal dominant disorder characterized by persistent blisters and suprabasal cell separation (acantholysis) of the epidermis, due to impaired keratinocyte adhesion. Patients lacking all isoforms except isoform 2 have HHD.
Subcellular location: Golgi apparatus membrane; Multi-pass membrane protein
Tissue specificity: Found in most tissues except colon, thymus, spleen and leukocytes. Most abundant in keratinocytes and kidney.
Defects in ATP2C1 are the cause of Hailey-Hailey disease (HHD); also known as benign familial pemphigus. HHD is an autosomal dominant disorder characterized by persistent blisters and suprabasal cell separation (acantholysis) of the epidermis, due to impaired keratinocyte adhesion. Patients lacking all isoforms except isoform 2 have HHD.
Subcellular location: Golgi apparatus membrane; Multi-pass membrane protein
Tissue specificity: Found in most tissues except colon, thymus, spleen and leukocytes. Most abundant in keratinocytes and kidney.
Storage
Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
Expiry Date
12 months after reconstitution
Shipping
This item will be shipped to you at ambient temperature in a lyophilised form.
References
1. Hu Z, et al. Nat. Genet. 24:61-65(2000)
2. Fairclough R.J, et al. J. Biol. Chem. 278:24721-24730(2003)
3. Dobson-Stone C, et al. J. Invest. Dermatol. 118:338-343(2002)
2. Fairclough R.J, et al. J. Biol. Chem. 278:24721-24730(2003)
3. Dobson-Stone C, et al. J. Invest. Dermatol. 118:338-343(2002)
Limitation
For research use only
Code
OSA00162W
$240USD
Unit size
100 µl
Conjugate
Unconjugated antibody
Host
NZ white rabbit
Purity
Whole serum
Clonality
Polyclonal
Isotype
Polyclonal, whole serum
Applications
IHC, WB (confirmed by recombinant protein). A dilution of 1: 300 to 1: 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.
Specificity
Specific for ATP2C1.
Spcs X-react.
Human, rat, mouse. Other species not yet tested.
Format
Lyophilised
Reconstitution
Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.
Note
Control peptide is available at $120 per 50 μg. Please enquire sales@osenses.com
Code
OSA00162W
$240USD
PDF Data Sheet
- Click to download the Data SheetMSDS
⚬ Products
Rabbit antibody to ATP2C1
