AIPL1, Monoclonal Antibody

Monoclonal AIPL1; Anti-AIPL1; Aryl hydrocarbon receptor interacting protein-like 1; LCA4; AIPL2; AIPL1; AIPL 1; AIPL-1

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

Monoclonal

IgG1

3B4

Mouse

AIPL1 antibody was purified by affinity chromatography.

Supplied in PBS buffer, pH 7.3, containing 1% BSA, 50% glycerol and 0.02% sodium azide.

500 ug-1 mg/ml (lot specific)

Store at 4 degree C for short term storage. Aliquot and store at -20 degree C for long term storage. Avoid repeated freeze/thaw cycles.

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.

Mouse monoclonal AIPL1 antibody

Cell Biology

Flow Cytometry (FC/FACS), Immunofluorescence (IF), Immunohistochemistry (IHC), Western Blot (WB)

IF: 1:100
FC: 1:100
IHC: 1:150
WB: 1:500-2000

Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant AIPL1 protein (Right) or empty vector (Left) detected with AIPL1 antibody

Immunohistochemical analysis of AIPL1 protein in paraffin embedded Carcinoma of Human thyroid tissue using AIPL1 antibody

Immunofluorescent staining of COS7 cells transiently transfected with recombinant AIPL1 protein using AIPL1 antibody

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: Chaperone; Apoptosis

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

Disease: Leber Congenital Amaurosis 4; Retinitis Pigmentosa

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