AIPL1, Polyclonal Antibody

Anti-AIPL1 Antibody (Internal) IHC-plus; AIPL1; AIPL2; LCA4; Human AIPL1

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

Polyclonal

Rabbit

At least three isoforms of Aipl1 are known to exist.

Immunoaffinity Purified

PBS containing 0.02% sodium azide

1 mg/ml (lot specific)

Short term 4 degree C, long term aliquot and store at -20 degree C, avoid freeze thaw cycles. Store undiluted.

Small volumes of anti-AIPL1 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.

Immunohistochemistry (IHC - Paraffin), Western Blot (WB), ELISA (EIA)

ELISA, IHC-P (5 ug/ml), WB (1 - 2 ug/ml)

Anti-AIPL1 antibody IHC of human adrenal. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.

Immunofluorescence of aipl1 in human brain tissue with aipl1 antibody at 20 ug/ml.

Western blot of Aipl1 in rat brain tissue lysate with Aipl1 antibody at (A) 1 and (B) 2 ug/ml.

40,901 Da

aryl hydrocarbon receptor interacting protein-like 1

aryl-hydrocarbon-interacting protein-like 1

Aryl-hydrocarbon-interacting protein-like 1

AIPL2  [Similar Products]

AIPL1_HUMAN

Leber congenital amaurosis (LCA) is the most severe inherited retinopathy with the earliest age of onset and accounts for at least 5% of all inherited retinal diseases. Affected individuals are diagnosed at birth or in the first few months of life with nystagmus, severely impaired vision or blindness and an abnormal or flat electroretinogram. The photoreceptor/pineal-expressed gene, AIPL1, encoding aryl-hydrocarbon interacting protein-like 1, is located within the LCA4 candidate region. The encoded protein contains three tetratricopeptide motifs, consistent with chaperone or nuclear transport activity. Mutations in this gene may cause approximately 20% of recessive LCA. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014]

AIPL1: May be important in protein trafficking and/or protein folding and stabilization. Defects in AIPL1 are the cause of Leber congenital amaurosis type 4 (LCA4). LCA designates a clinically and genetically heterogeneous group of childhood retinal degenerations, generally inherited in an autosomal recessive manner. Affected infants have little or no retinal photoreceptor function as tested by electroretinography. LCA represents the most common genetic cause of congenital visual impairment in infants and children. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Apoptosis; Chaperone

Chromosomal Location of Human Ortholog: 17p13.1

Cellular Component: photoreceptor inner segment; cytoplasm; nucleus

Molecular Function: farnesylated protein binding; protein binding; unfolded protein binding

Biological Process: retinal homeostasis; phototransduction, visible light; protein farnesylation; protein folding; visual perception; regulation of cGMP metabolic process; negative regulation of apoptosis

Disease: Leber Congenital Amaurosis 4; Retinitis Pigmentosa

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.