ABCA4 transports NRPE from photoreceptor outer segment membrane to cytosol

Stable Identifier
Reaction [transition]
Homo sapiens
NRPE is a substrate for ABCA4
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Bovine studies (Beharry et al. 2004) have indicated NRPE to be the preferred substrate for ABCA4 (ABCR, rim protein, RmP) (Azarian et al. 1998), which acts as an inward-directed retinoid flippase and facilitates the transfer of NRPE to the cytoplasmic side of the disc membrane. This transfer is essential to avoid build up of potentially toxic retinoid intermediates which are implicated in many retinal degenerative diseases (see review Tsybovsky et al. 2010). Defective ABCA4 cannot perform this function leading to impaired vision and blindness disorders such as Stargardt disease (MIM:248200).

Literature References
PubMed ID Title Journal Year
15471866 N-retinylidene-phosphatidylethanolamine is the preferred retinoid substrate for the photoreceptor-specific ABC transporter ABCA4 (ABCR)

Beharry, S, Zhong, M, Molday, RS

J. Biol. Chem. 2004
9703434 The human photoreceptor rim protein gene (ABCR): genomic structure and primer set information for mutation analysis

Megarity, CF, Azarian, SM, Travis, GH, Horvath, DH, Weng, J

Hum. Genet. 1998
Catalyst Activity

retinol transmembrane transporter activity of ABCA4 [photoreceptor disc membrane]

Inferred From
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