Rabbit anti-Human, Mouse Aipl1 Polyclonal Antibody | anti-Aipl1 antibody

Aipl1 Antibody

Cat. Num. AAA9407874

• Gene Names: AIPL1; LCA4; AIPL2
• Reactivity: Human, Mouse
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: Affinity chromatography purified via peptide column
• Synonyms: Aipl1; Polyclonal Antibody; Aipl1 Antibody; Aryl hydrocarbon receptor interacting protein-like 1; LCA4; anti-Aipl1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Purity/Purification: Affinity chromatography purified via peptide column
• Form/Format: Supplied in PBS containing 0.02% sodium azide.
• Sequence Length: 384

• Applicable Applications for anti-Aipl1 antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Immunogen Type: Peptide
• Immunogen Description: Raised against a 17 amino acid peptide near the carboxy terminus of the human Aipl1.
• Target Name: Aipl1
• Preparation and Storage: Can be stored at -20 degree C, stable for 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.

Western Blot (WB)

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

Immunohistochemistry (IHC)

(Immunohistochemistry of Aipl1 in human brain tissue with Aipl1 antibody at 2.5 ug/mL.)

Related Product Information for anti-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.

Product Categories/Family for anti-Aipl1 antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 74272276
• NCBI GeneID: 23746
• NCBI Accession #: NP_055151
• NCBI GenBank Nucleotide #: NM_014336.4
• Molecular Weight: 40,901 Da
• NCBI Official Full Name: aryl-hydrocarbon-interacting protein-like 1 isoform 1
• NCBI Official Synonym Full Names: aryl hydrocarbon receptor interacting protein-like 1
• NCBI Official Symbol: AIPL1
• NCBI Official Synonym Symbols: LCA4; AIPL2
• NCBI Protein Information: aryl-hydrocarbon-interacting protein-like 1
• UniProt Protein Name: Aryl-hydrocarbon-interacting protein-like 1
• Protein Family: Aryl-hydrocarbon-interacting protein
• UniProt Gene Name: AIPL1
• UniProt Synonym Gene Names: AIPL2
• UniProt Entry Name: AIPL1_HUMAN

NCBI Description

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]

Uniprot Description

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