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Anti-ABCA4

Produktgrößen
100 ul
£587,00
OAPC00010-100UL
Über dieses Produkt
SKU:
OAPC00010
Zusätzliche Namen:
R; RmP; Abc1; Abcr; Abc10; AW050280; D430003I15Rik
translate.label.attr.clone:
3F4
Klonalität:
Monoclonal
Weitere Details:
ABCA4 (ATP-binding cassette; sub-family A (ABC1); member 4; Rim Protein) is a member of the superfamily of ATP-binding cassette (ABC) transporters (Illing et al.; 1997). ABC proteins transport various molecules across extra- and intracellular membranes. This protein is a retina-specific ABC transporter with N-retinylidene-PE as a substrate. It is expressed exclusively in retina photoreceptor cells; indicating the gene product mediates transport of an essential molecule across the photoreceptor cell membrane. Mutations in this gene are found in patients diagnosed with Stargardt disease and are associated with retinitis pigmentosa-19 and age-related macular degeneration (Wiszniewski et al.; 2003). Defects in ABCA4 are the cause of Stargardt disease type 1 (STGD1) (Molday et al.; 2000). STGD is one of the most frequent causes of macular degeneration in childhood. Defects in ABCA4 are also known to cause fundus flavimaculatus (FFM); age-related macular degeneration type 2 (ARMD2) and cone-rod dystrophy type 3 (CORD3) (Klevering et al.; 2005).
Host:
Mouse
Immunogen:
Partially purified bovine 220-kDa disc rim protein.
Isotyp:
IgG
Molekulargewicht:
220 kDa
Proteindetails:
Retinal-specific ATP-binding cassette transporter
Aufreinigung:
Protein G purified culture supernatant.
Versandbedingungen:
Blue Ice
Lagerbedingungen:
Store at -20C in undiluted aliquots; stable for up to one year after date of receipt. Avoid freeze/thaw cycles.
Hersteller:
Aviva Systems Biology
Typ:
Proteins, Peptides, Small Molecules & Other Biomolecules