| Description | |
|---|---|
| Catalogue Number | ABN1441 |
| Description | Anti-ABCA4 |
| Alternate Names |
|
| Background Information | Retinal-specific ATP-binding cassette transporter (UniProt: P78363; also known as ATP-binding cassette sub-family A member 4, RIM ABC transporter, RIM protein, RmP, Stargardt disease protein) is encoded by the ABCA4 (also known as ABCR) gene (Gene ID: 24) in human. ABC transporter A4 is a multi-pass membrane protein of the superfamily of ATP-binding cassette (ABC) transporters that is localized to the disk margins of vertebrate photoreceptor outer segments. It is implicated in the clearance of all-trans retinal derivatives from rod and cone photoreceptor cells following photoexcitation as part of the visual cycle. ABC transporter A4 is composed of two symmetrically organized halves, each comprising six membrane-spanning helices, a large glycosylated exocytoplasmic domain located inside the disk, and a cytoplasmic domain with an ATP-binding cassette. Mutations in ABCA4 gene are known to cause Stargardt macular degeneration, which is an autosomal recessive form of juvenile macular degeneration associated with a severe loss in vision. Mutations in ABCA4 gene are also known to cause retinitis pigmentosa, which is characterized by retinal pigment deposits and loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. (Ref.: Tsybovsky, Y., et al. (2010). Adv. Exp. Med. Biol. 703, 105-125). |
| Product Information | |
|---|---|
| Format | Affinity Purified |
| Presentation | Purified rabbit polyclonal antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-ABCA4, Cat. No. ABN1441, is a highly specific rabbit polyclonal antibody that targets Retinal-specific ATP-binding cassette transporter (ABCA4) and has been tested for use in Immunohistochemistry (Paraffin) and Western Blotting. |
| Key Applications |
|
| Application Notes | Immunohistochemistry Analysis: A 1:1,000 dilution from a representative lot detected ABCA4 in human retina tissue. |
| Biological Information | |
|---|---|
| Immunogen | GST-tagged reombinant fragment corresponding to 112 amino acids from the internal region of human Retinal-specific ATP-binding cassette transporter (ABCA4). |
| Concentration | Please refer to lot specific datasheet. |
| Host | Rabbit |
| Specificity | This rabbit polyclonall antibody detects Retinal-specific ATP-binding cassette transporter (ABCA4) in Human retina. It targets an epitope within 112 amino acids from the cytoplasmic domain. |
| Species Reactivity |
|
| Species Reactivity Note | Human. |
| Antibody Type | Polyclonal Antibody |
| Entrez Gene Number |
|
| Gene Symbol |
|
| Purification Method | Affinity Purified |
| UniProt Number |
|
| Molecular Weight | ~256 kDa observed; 255.94 kDa calculated. Uncharacterized bands may be observed in some lysate(s). |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Evaluated by Western Blotting in human retina tissue lysate. Western Blotting Analysis: 2 µg/mL of this antibody detected ABCA4 in 10 µg of human retina tissue lysate. |
| Usage Statement |
|
| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 100 μg |