ELISA/assay

Human Aryl hydrocarbon interacting protein like 1 (AIPL1) ELISA Kit

MBS7205041

48-Strip-Wells

470 USD

ELISA Kit

Human Aryl hydrocarbon interacting protein like 1 (AIPL1) ELISA Kit

Aryl hydrocarbon interacting protein like 1 (AIPL1)

Aryl-hydrocarbon-interacting protein-like 1; Aryl-hydrocarbon-interacting protein-like 1; aryl-hydrocarbon-interacting protein-like 1; aryl hydrocarbon receptor interacting protein-like 1

AIPL1

AIPL1; AIPL1; LCA4; AIPL2; AIPL2

AIPL1_HUMAN

Human

This assay has high sensitivity and excellent specificity for detection of AIPL1. No significant cross-reactivity or interference between AIPL1 and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between AIPL1 and all the analogues, therefore, cross reaction may still exist in some cases.

Store all reagents at 2-8 degree C.

Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Assay Type: Competitive. Sensitivity: 0.1 ng/mL.

Intended Uses: This AIPL1 ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human AIPL1. This ELISA kit for research use only, not for therapeutic or diagnostic applications!

Neurobiology

Principle of the assay: AIPL1 ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-AIPL1 antibody and an AIPL1-HRP conjugate. The assay sample and buffer are incubated together with AIPL1-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the AIPL1 concentration since AIPL1 from samples and AIPL1-HRP conjugate compete for the anti-AIPL1 antibody binding site. Since the number of sites is limited, as more sites are occupied by AIPL1 from the sample, fewer sites are left to bind AIPL1-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The AIPL1 concentration in each sample is interpolated from this standard curve.

23503042

Q9NZN9.2

40,901 Da

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: 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

48-Strip-Wells

470 USD

96-Strip-Wells

675