Functional Interaction between the Two Halves of the Photoreceptor-specific ATP Binding Cassette Protein ABCR (ABCA4)

ABCR, also known as ABCA4, is a member of the superfamily of ATP binding cassette transporters that is believed to transport retinal or retinylidene-phosphatidylethanolamine across photoreceptor disk membranes. Mutations in the ABCR gene are responsible for Stargardt macular dystrophy retinal that in ABCR of a a a a binding the of the binding of the of ABCR in ABCR in disk membranes. disk or the in the the to that the ATP ABCR in that of ABCR ATP in the or of a to a in ABCR ABCR, also known as ABCA4, is a member of the superfamily of ATP binding cassette transporters that is believed to transport retinal or retinylidene-phosphatidylethanolamine across photoreceptor disk membranes. Mutations in the ABCR gene are responsible for Stargardt macular dystrophy retinal that in ABCR of a a a a binding the of the binding of the of ABCR in ABCR in disk membranes. disk or the in the the to that the ATP ABCR in that of ABCR ATP in the or of a to a in ABCR ABCR, known as the is a member of the of ATP binding cassette ATP binding binding ATP binding binding transporters that is the of photoreceptor disk is to in the transport of or of across the disk the of transport to to the of disk as a in the of to retinal for of the of retinal retinylidene-phosphatidylethanolamine in disk that to of ABCR in photoreceptor is the that in the ABCR gene a of retinal Stargardt macular Stargardt macular the in ABCR, is of of known as the of macular of the retinal of of the in retinal photoreceptor transporters of that as a for the of a across ATP binding or binding that the for transport are a as in the of the or as a of or as in the ABCR in the of a of a of ABCR of the is the of a that the to the in of the ATP binding of for a of transporters of or the of the of the ATP binding of is for transport the of is the is or the the is the of as a also the of of binding also a of the ATP binding ATP that the binding of for of the binding of the in of the for of the the of of ABCR, the of the or in of ABCR in disk membranes. that the of ABCR to a to in the or to a that a the the of ATP ABCR ATP to transport across photoreceptor disk the of ABCR, the to the of ABCR the to for the of ABCR as a the to the are in the for the are in the that the the in the of the the the the the of that of the the the to the of for the of the of a the for the of the the for the in the of in in the the of the in for the to of for a in a in of of of for of of the for of as the of as a of the the is of of that in the the that in the of as the the or a a of for of in of or for a of the in in to of in the of to for in in in of or for the of the to in the of in the in of for to as for of as for the of of for to as for a of to the the for in of of the the as in of of the for as in of in a for of a of to the as to of the to as of of to ABCR or in ABCR to for a of ABCR in in a for as the as to the of ATP ABCR for in a for known of ATP as to the of of of ATP of for ATP for to to ATP of as of in ATP to for for ATP for ATP of to a of to of ATP in the to known of ATP in the the of to ATP the of ATP of to the of ATP the that the the in the a of to the of the ABCR the of the the of ATP ABCR, the ATP ABCR in in to a for of ABCR in of ABCR the in in the is for ABCR of of in the in to a in ABCR the to a for ABCR a of for for for of the or of the a of the in in of ABCR of ABCR, a or in of of the ABCR the a the of the the the the the in the to the the the to that ABCR the in the the of ABCR in a the the in a a of the are also the ABCR, or the the a ABCR the the ABCR of the ABCR are the the of retinal of ABCR the or in that the ABCR a of the the the of the a for retinal the of the of the as of the of ABCR are for ABCR or in or in as a of the are of to Mutations ATP in the of is for the of of to in ABCR, the to in the or in the of ABCR retinal also the the that of the to in ABCR ATP ATP in the or of in in a ATP or a the the ATP binding of the ATP of the to ABCR to the of ABCR in for the the ATP to or of ABCR, ABCR that the the the of ATP to the ATP the of ATP of the of ABCR or the in the or of a to ABCR, the the or as of in of the as or ABCR the a of the ABCR the for the ABCR the the the or for for or ABCR the of ABCR also ABCR in disk to of for the as in ABCR to also that the is is of that the of or the of is responsible for ATP the or is for ATP that ATP binding the of ABCR to the ATP binding of a member of the that is to that the of in to binding to the of the of to the in the ABCR of of ABCR a ABCR a are the of the are the the of the of ABCR is also the that is is to a that that is that the of ABCR the of ABCR that is to the the of the of the of ABCR, in ABCR binding or the of of the that as ATP in of the ABCR of ABCR of retinal ABCR ABCR ABCR in the of in the of of of that of ABCR or of that a ABCR a the of ABCR the ATP in ABCR of of in the of the of the of ABCR in a in the of also or of the ATP binding of or of ABCR of ABCR ABCR in a ABCR the of of of ABCR, the of that ABCR of that ABCR of ABCR in to in the the of ABCR, that is to as as the that the is to also in the of the for ABCR retinal ATP to the binding of in that the of ABCR a of the of to are of ABCR in in the is of ABCR in in the of of ABCR in the of retinal of of of of of of of of ABCR that the is to to of or ABCR to the the that the of ABCR of ABCR in the of known to the also the of ABCR that the a that or the ATP the of the of ABCR the in ABCR in membranes. to the the a that is a as for the is in ABCR the also of a of the ABCR, the to that is as to the of the the the the of in the the or is the that the are that are in the in as as the also to in the for transporters ATP to the transport of across is in the of the are in the are for ATP the ATP of the to transport ATP in a to ATP the is to to of transporters that the of transporters binding the of is a of ATP ATP is ATP binding to the binding of ATP of that in a the ATP is for transport across ATP is of ABCR, of are in of ABCR the that ABCR transporters in ABCR in the in the of the retinal in the of the of a of is the for the of of ABCR to is of the of the to that in is of ATP the also binding to of the of a the of ABCR or that are known to in of that or the in of ABCR in the of the of to ABCR the binding of to that in ABCR a the of is the is to ATP binding is the of ABCR the in the that the of are of the for the is the that the ATP the of that is that of to that the is in a that the in a for the of ATP to the of of a in is responsible for of ABCR, in the to in is that in the binding of are of ABCR that is the of ATP binding in the of retinal the to a that a in the of Mutations in of ABCR the of in in a a of ABCR of in a of the that Mutations in the a in ATP binding is responsible for in that of the of ABCR in a in gene for Stargardt macular a to retinal retinal a of ABCR a of to the the a photoreceptor in photoreceptor in a that or the of Stargardt macular ABCR, known as the is a member of the of ATP binding cassette ATP binding binding ATP binding binding transporters that is the of photoreceptor disk is to in the transport of or of across the disk the of transport to to the of disk as a in the of to retinal for of the of retinal retinylidene-phosphatidylethanolamine in disk that to of ABCR in photoreceptor is the that in the ABCR gene a of retinal Stargardt macular Stargardt macular the in ABCR, is of of known as the of macular of the retinal of of the in retinal photoreceptor transporters of that as a for the of a across ATP binding or binding that the for transport are a as in the of the or as a of or as in the ABCR in the of a of a of ABCR of the is the of a that the to the in of the ATP binding of for a of transporters of or the of the of the ATP binding of is for transport the of is the is or the the is the of as a also the of of binding also a of the ATP binding ATP that the binding of for of the binding of the in of the for of the the of of ABCR, the of the or in of ABCR in disk membranes. that the of ABCR to a to in the or to a that a the the of ATP ABCR ATP to transport across photoreceptor disk membranes. the of ABCR, the to the of ABCR the to for the of ABCR as a the to the are in the for the are in the that the the in the of the the the the the of that of the the the to the of for the of the of a the for the of the the for the in the of in in the the of the in for the to of for a in a in of of of for of of the for of as the of as a of the the is of of that in the the that in the of as the the or a a of for of in of or for a of the in in to of in the of to for in in in of or for the of the to in the of in the in of for to as for of as for the of of for to as for a of to the the for in of of the the as in of of the for as in of in a for of a of to the as to of the to as of of to ABCR or in ABCR to for a of ABCR in in a for as the as to the of ATP ABCR for in a for known of ATP as to the of of of ATP of for ATP for to to ATP of as of in ATP to for for ATP for ATP of to a of to of ATP in the to known of ATP in the the of to ATP the of ATP of to the of ATP the that the the in the a of to the of the ABCR the of the the of ATP ABCR, the ATP ABCR in in to a for the of ABCR, the to the of ABCR the to for the of ABCR as a the to the are in the for the are in the that the the in the of the the the the the of that of the the the to the of for the of the of a the for the of the the for the in the of in in the the of the in for the to of for a in a in of of of for of of the for of as the of as a of the the is of of that in the the that in the of as the the or a a of for of in of or for a of the in in to of in the of to for in in in of or for the of the to in the of in the in of for to as for of as for the of of for to as for a of to the the for in of of the the as in of of the for as in of in a for of a of to the as to of the to as of of to ABCR or in ABCR to for a of ABCR in in a for as the as to the of ATP ABCR for in a for known of ATP as to the of of of ATP of for ATP for to to ATP of as of in ATP to for for ATP for ATP of to a of to of ATP in the to known of ATP in the the of to ATP the of ATP of to the of ATP the that the the in the a of to the of the ABCR the of the the of ATP ABCR, the ATP ABCR in in to a for of ABCR in of ABCR the in in the is for ABCR of of in the in to a in ABCR the to a for ABCR a of for for for of the or of the a of the in in of ABCR of ABCR, a or in of of the ABCR the a the of the the the the the in the to the the the to that ABCR the in the the of retinal of ABCR the or in that the ABCR a of the the the of the a for retinal the of the of the as of the of ABCR are for ABCR or in or in as a of the are of to Mutations ATP in the of is for the of of to in ABCR, the to in the or in the of ABCR retinal also the the that of the to in ABCR ATP ATP in the or of in in a ATP or a the the ATP binding of the ATP of the to ABCR to the of ABCR in for the the ATP to or of ABCR, ABCR that the the the of ATP to the ATP the of ATP of the of ABCR or the in the or of a to ABCR, the the or as of in of the as or ABCR the a of the ABCR the for the ABCR the the the or for for or ABCR the of ABCR also ABCR in disk to of for the as in ABCR to also that the is is of that the of or the of is responsible for ATP the or is for ATP that ATP binding the of ABCR to the ATP binding of a member of the that is to that the of in to binding to the of the of to the in the ABCR of of ABCR a ABCR a are the of the are the the of the of ABCR is also the that is is to a that that is that the of ABCR the of ABCR that is to the the of the of the of ABCR, in ABCR binding or the of of the that as ATP in of the ABCR of ABCR of retinal ABCR ABCR ABCR in the of in the of of of that of ABCR or of that a ABCR a the of ABCR the ATP in ABCR of of in the of the of the of ABCR in a in the of also or of the ATP binding of or of ABCR of ABCR ABCR in a ABCR the of of of ABCR, the of that ABCR of that ABCR of ABCR in to in the the of ABCR, that is to as as the that the is to also in the of the for ABCR retinal ATP to the binding of in that the of ABCR a of the of to are of ABCR in in the is of ABCR in in the of of ABCR in the of retinal of of of of of of of of ABCR that the is to to of or ABCR to the the that the of ABCR of ABCR in the of known to the also the of ABCR that the a that or the ATP of ABCR in of ABCR the in in the is for ABCR of of in the in to a in ABCR the to a for ABCR a of for for for of the or of the a of the in in of ABCR of ABCR, a or in of of the ABCR the a the of the the the the the in the to the the the to that ABCR the in the the of retinal of ABCR the or in that the ABCR a of the the the of the a for retinal the of the of the as of the of to Mutations ATP in the of is for the of of to in ABCR, the to in the or in the of ABCR retinal also the the that of the the ATP binding of the ATP of the to ABCR to the of ABCR in for the the ATP to or of ABCR, ABCR that the the the of ATP to the ATP the of ATP the of ABCR also ABCR in disk to of for the as in ABCR to also that the is is of that the of or the of is responsible for ATP the or is for ATP that ATP binding the of ABCR to the ATP binding of a member of the that is to that the of in to also the that is is to a that that is that the of ABCR the of ABCR that is to the the of the of the of ABCR, in ABCR binding or the of of the that as ATP in of the of of that of ABCR or of that a ABCR a the of ABCR the ATP in ABCR of of in the of the of the of ABCR in a in the of also or of the ATP binding of or ABCR the of of of ABCR, the of that ABCR of that ABCR of ABCR in to in the the of ABCR, that is to as as the that the is to also in the of the for ABCR retinal ATP to the binding of in that the of ABCR a to to of or ABCR to the the that the of ABCR of ABCR in the of known to the also the of ABCR that the a that or the ATP the of the of ABCR the in ABCR in membranes. to the the a that is a as for the is in ABCR the also of a of the ABCR, the to that is as to the of the the the the of in the the or is the that the are that are in the in as as the also to in the for transporters ATP to the transport of across is in the of the are in the are for ATP the ATP of the to transport ATP in a to ATP the is to to of transporters that the of transporters binding the of is a of ATP ATP is ATP binding to the binding of ATP of that in a the ATP is for transport across ATP is of ABCR, of are in of ABCR the that ABCR transporters in ABCR in the in the of the retinal in the of the of a of is the for the of of ABCR to is of the of the to that in is of ATP the also binding to of the of a the of ABCR or that are known to in of that or the in of ABCR in the of the of to ABCR the binding of to that in ABCR a the of is the is to ATP binding is the of ABCR the in the that the of are of the for the is the that the ATP the of that is that of to that the is in a that the in a for the of ATP to the of of a in is responsible for of ABCR, in the to in is that in the binding of are of ABCR that is the of ATP binding in the of retinal the to a that a in the of Mutations in of ABCR the of in in a a of ABCR of in a of the that Mutations in the a in ATP binding is responsible for in that of the of ABCR in a in gene for Stargardt macular a to retinal retinal a of ABCR a of to the the a photoreceptor in photoreceptor in a that or the of Stargardt macular the of the of ABCR the in ABCR in membranes. to the the a that is a as for the is in ABCR the also of a of the ABCR, the to that is as to the of the the the the of in the the or is the that the are that are in the in as as the also to in the for transporters ATP to the transport of across is in the of the are in the are for ATP the ATP of the to transport ATP in a to ATP the is to to of transporters that the of transporters binding the of is a of ATP ATP is ATP binding to the binding of ATP of that in a the ATP is for transport across ATP is of ABCR, of are in of ABCR the that ABCR transporters in ABCR in the in the of the retinal in the of the of a of is the for the of of ABCR to is of the of the to that in is of ATP the also binding to of the of a the of ABCR or that are known to in of that or the in of ABCR in the of the of to ABCR the binding of to that in ABCR a the of is the is to ATP binding is the of ABCR the in the that the of are of the for the is the that the ATP the of that is that of to that the is in a that the in a for the of ATP to the of of a in is responsible for of ABCR, in the to in is that in the binding of are of ABCR that is the of ATP binding in the of retinal the to a that a in the of Mutations in of ABCR the of in in a a of ABCR of in a of the that Mutations in the a in ATP binding is responsible for in that of the of ABCR in a in gene for Stargardt macular a to retinal retinal a of ABCR a of to the the a photoreceptor in photoreceptor in a that or the of Stargardt macular for of the in

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