LCA14

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

Polyclonal

IgG

Rabbit

Affinity Purification

Store at -20 degree C (regular) or -80 degree C (long term). Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Manufactured in an ISO 9001:2015 Certified Laboratory.

Small volumes of anti-LRAT  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.

The protein encoded by this gene localizes to the endoplasmic reticulum, where it catalyzes the esterification of all-trans-retinol into all-trans-retinyl ester. This reaction is an important step in vitamin A metabolism in the visual system. Mutations in this gene have been associated with early-onset severe retinal dystrophy and Leber congenital amaurosis 14. Alternative splicing results in multiple transcript variants.

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

WB: 1:500-1:2000, IHC: 1:50-1:200, IF: 1:50-1:100

Immunofluorescence analysis of U2OS cell using LRAT antibody. Blue: DAPI for nuclear staining.

Calculated MW: 25kDa

lecithin retinol acyltransferase (phosphatidylcholine--retinol O-acyltransferase)

lecithin retinol acyltransferase

Lecithin retinol acyltransferase

LRAT_HUMAN

The protein encoded by this gene localizes to the endoplasmic reticulum, where it catalyzes the esterification of all-trans-retinol into all-trans-retinyl ester. This reaction is an important step in vitamin A metabolism in the visual system. Mutations in this gene have been associated with early-onset severe retinal dystrophy and Leber congenital amaurosis 14. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2014]

LRAT: Transfers the acyl group from the sn-1 position of phosphatidylcholine to all-trans retinol, producing all-trans retinyl esters. Retinyl esters are storage forms of vitamin A. LRAT plays a critical role in vision. It provides the all-trans retinyl ester substrates for the isomerohydrolase which processes the esters into 11-cis-retinol in the retinal pigment epithelium; due to a membrane-associated alcohol dehydrogenase, 11 cis-retinol is oxidized and converted into 11-cis-retinaldehyde which is the chromophore for rhodopsin and the cone photopigments. Defects in LRAT are a cause of Leber congenital amaurosis type 14 (LCA14). It is a severe dystrophy of the retina, typically becoming evident in the first years of life. Visual function is usually poor and often accompanied by nystagmus, sluggish or near-absent pupillary responses, photophobia, high hyperopia and keratoconus. Belongs to the H-rev107 family.

Protein type: EC 2.3.1.135; Transferase; Membrane protein, integral; Cofactor and Vitamin Metabolism - retinol

Chromosomal Location of Human Ortholog: 4q32.1

Cellular Component: endoplasmic reticulum membrane; integral to membrane; multivesicular body; perinuclear region of cytoplasm; rough endoplasmic reticulum

Molecular Function: phosphatidylcholine-retinol O-acyltransferase activity; retinoic acid binding; retinol binding; transferase activity, transferring acyl groups

Biological Process: fat-soluble vitamin metabolic process; phototransduction, visible light; positive regulation of lipid transport; retinoic acid metabolic process; retinoid metabolic process; retinol metabolic process; visual perception; vitamin A metabolic process; vitamin metabolic process

Disease: Leber Congenital Amaurosis 14; 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.