antibody

Aipl1 Antibody

MBS150349

0.1 mg

345 USD

polyclonal

rabbit

nonconjugated

human, mouse

western blot, ELISA, immunohistochemistry, immunocytochemistry, enzyme immunoassay

Antibody

Aipl1 Antibody

Aipl1

Aipl1; LCA4; AIPL2; Aryl-hydrocarbon-interacting protein-like 1; aryl hydrocarbon receptor interacting protein-like 1

aryl-hydrocarbon-interacting protein-like 1 isoform 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

Polyclonal

IgG

Rabbit

Human, Mouse

384

Aipl1 Antibody is affinity chromatography purified via peptide column.

Liquid

1 mg/mL

Aipl1 antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

Aipl1 antibody can be used for detection of Aipl1 by Western blot at 1 - 2 mug/mL. Antibody can also be used for immunohistochemistry starting at 2.5 mug/mL. For immunofluorescence start at 20 mug/mL.

Conjugate: Unconjugated. Immunogen: Aipl1 antibody was raised against a 17 amino acid synthetic peptide near the carboxy terminus of the human Aipl1. Buffer: Aipl1 Antibody is supplied in PBS containing 0.02% sodium azide.

Aipl1 Antibody: Aipl1 was initially identified as a protein implicated in Leber congenital amaurosis (LCA), an autosomal recessive disorder thought to be caused by the abnormal development of photoreceptors. Aipl1 is a tetratricopeptide repeat protein that is highly homologous to ARA9, a protein involved in the HSP90-mediated nuclear translocation and transactivation of the aryl hydrocarbon receptor. Aipl1 has also been found to function as part of a chaperone heterocomplex, interacting with Hsp90 and Hsp70. Aipl1 also associates with the cell cycle regulator NUB1. It is thought that Aipl1 cooperates with Hsp70 but not Hsp90 to suppress the formation of NUB1 inclusions, and these interactions are necessary in the normal photoreceptor maturation, as mutations that lead to LCA also compromise the interactions with the Hsp chaperones. At least three isoforms of Aipl1 are known to exist.

74272276

NP_055151

NM_014336.4

Q9NZN9

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]

0.1 mg

345 USD