Retinal-specific ATP-binding cassette transporter; ATP-binding cassette, sub-family A(ABC1), member 4; ABC 10 antibody; ABC A4 antibody; ABC10 antibody; ABCA 4 antibody; abcA4 antibody; ABCA4_HUMAN antibody; ABCR antibody; ARMD 2 antibody; ARMD2 antibody; ATP binding cassette 10 antibody; ATP binding cassette sub family A member 4 antibody; ATP binding cassette sub family A member4 antibody; ATP binding cassette transporter antibody; ATP binding cassette transporter retinal specific antibody; ATP binding cassette, sub family A(ABC1), member 4 antibody; ATP binding cassette, sub family A(ABC1), member4 antibody; ATP binding cassette10 antibody; ATP binding transporter, retina specific antibody; ATP-binding cassette sub-family A member 4 antibody; CORD 3 antibody; CORD3 antibody; DKFZp781N1972 antibody; FFM antibody; FLJ17534 antibody; Photoreceptor rim protein antibody; Retina specific ABC transporter antibody; Retinal specific ATP binding cassette transporter antibody; Retinal-specific ATP-binding cassette transporter antibody; RIM ABC transporter antibody; RIM protein antibody; RmP antibody; RP 19 antibody; RP19 antibody; Stargardt disease protein antibody; STGD antibody; STGD1 antibody

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Immunogen affinity purified.

Lyophilized

At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-ABCA4 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Description: Rabbit IgG polyclonal antibody for Retinal-specific ATP-binding cassette transporter(ABCA4) detection. Tested with WB in Mouse, Rat.
Background: ABCA4(ATP-Binding Cassette, Subfamily A, Member 4), also known as ABCR, is a protein which in humans is encoded by the ABCA4 gene. ABCA4 is a member of the ATP-binding cassette transporter gene sub-family A(ABC1) found exclusively in multicellular eukaryotes. Using a whole genome radiation hybrid panel, Allikmets et al.(1997) mapped the ABCR gene to 1p21-p13. Allikmets et al.(1997) localized ABCR transcripts exclusively within photoreceptor cells, indicating that ABCR mediates the transport of an essential molecule(or ion) either into or out of photoreceptor cells. Molday et al.(2000) showed by immunofluorescence microscopy and Western blot analysis that ABCR is present in foveal and peripheral cone, as well as rod, photoreceptors. The results suggested that the loss in central vision experienced by patients with Stargardt macular dystrophy arises directly from ABCR-mediated foveal cone degeneration.

Western Blot (WB)

Anti-ABCA4 antibody, MBS176164, Western blotting
All lanes: Anti ABCA4 (MBS176164) at 0.5ug/ml
WB: Mouse Brain Tissue Lysate at 50ug
Predicted bind size: 256KD
Observed bind size: 256KD

260,209 Da

ATP-binding cassette, sub-family A (ABC1), member 4

retinal-specific ATP-binding cassette transporter; Rim protein; RIM ABC transporter; ATP-binding cassette 10; ATP-binding cassette sub-family A member 4

Retinal-specific ATP-binding cassette transporter

Abcr; RIM protein; RmP??[Similar Products]

ABCA4_MOUSE

The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intracellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ABC1 subfamily. Members of the ABC1 subfamily comprise the only major ABC subfamily found exclusively in multicellular eukaryotes. This protein was the first of the ABC transporters to be observed in photoreceptors and may play a role in the photoresponse. Mutations in the human gene are found in patients diagnosed with Stargardt disease and are associated with retinitis pigmentosa-19 and macular degeneration age-related 2. [provided by RefSeq, Jul 2008]

ABCA4: In the visual cycle, acts as an inward-directed retinoid flipase, retinoid substrates imported by ABCA4 from the extracellular or intradiscal (rod) membrane surfaces to the cytoplasmic membrane surface are all-trans-retinaldehyde (ATR) and N-retinyl-phosphatidyl-ethanolamine (NR-PE). Once transported to the cytoplasmic surface, ATR is reduced to vitamin A by trans- retinol dehydrogenase (tRDH) and then transferred to the retinal pigment epithelium (RPE) where it is converted to 11-cis-retinal. May play a role in photoresponse, removing ATR/NR-PE from the extracellular photoreceptor surfaces during bleach recovery. Defects in ABCA4 are the cause of Stargardt disease type 1 (STGD1). STGD is one of the most frequent causes of macular degeneration in childhood. It is characterized by macular dystrophy with juvenile-onset, rapidly progressive course, alterations of the peripheral retina, and subretinal deposition of lipofuscin-like material. STGD1 inheritance is autosomal recessive. Defects in ABCA4 are the cause of fundus flavimaculatus (FFM). FFM is an autosomal recessive retinal disorder very similar to Stargardt disease. In contrast to Stargardt disease, FFM is characterized by later onset and slowly progressive course. Defects in ABCA4 may be a cause of age-related macular degeneration type 2 (ARMD2). ARMD is a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid (known as drusen) that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Defects in ABCA4 are the cause of cone-rod dystrophy type 3 (CORD3). CORDs are inherited retinal dystrophies belonging to the group of pigmentary retinopathies. CORDs are characterized by retinal pigment deposits visible on fundus examination, predominantly in the macular region, and initial loss of cone photoreceptors followed by rod degeneration. This leads to decreased visual acuity and sensitivity in the central visual field, followed by loss of peripheral vision. Severe loss of vision occurs earlier than in retinitis pigmentosa. Defects in ABCA4 are the cause of retinitis pigmentosa type 19 (RP19). RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. RP19 is characterized by choroidal atrophy. Inheritance is autosomal recessive. Belongs to the ABC transporter superfamily. ABCA family.

Protein type: Membrane protein, multi-pass; Transporter, ABC family; Transporter; Membrane protein, integral

Cellular Component: photoreceptor outer segment; membrane; intracellular membrane-bound organelle; integral to plasma membrane; integral to membrane

Molecular Function: phospholipid-translocating ATPase activity; phospholipid transporter activity; transporter activity; ATPase activity, coupled to transmembrane movement of substances; cholesterol transporter activity; ATPase activity; nucleotide binding; ATP binding

Biological Process: phospholipid translocation; phospholipid transfer to membrane; visual perception; phospholipid efflux; transport; response to stimulus; photoreceptor cell maintenance; cholesterol efflux; transmembrane transport

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