anti-gcs1 antibody product blog
Tags: Antibody; Polyclonal Antibody; GCS1; anti-gcs1 antibody;
The gcs1 gcs1 (Catalog #MBS624030) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The GCS1, NT (Mannosyl-oligosaccharide Glucosidase, Processing A-glucosidase I, MOGS) reacts with Human and may cross-react with other species as described in the data sheet. MyBioSource\'s GCS1 can be used in a range of immunoassay formats including, but not limited to, ELISA (EL/EIA), Western Blot (WB).Suitable for use in ELISA and Western Blot.
Dilution: ELISA: 1:1,000
Western Blot: 1:100-1:500. Researchers should empirically determine the suitability of the gcs1 gcs1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process.
The gcs1 gcs1 product has the following accession number(s) (GI #19113943) (NCBI Accession #NP_593031.1) (Uniprot Accession #Q09768). Researchers may be interested in using Bioinformatics databases such as those available at The National Center for Biotechnology Information (NCBI) website for more information about accession numbers and the proteins they represent. Even researchers unfamiliar with bioinformatics databases will find the NCBI databases to be quite user friendly and useful.
To buy or view more detailed product information and pricing, please click on the technical datasheet page below:
Please refer to the product datasheet for known applications of a given antibody. We\'ve tested the GCS1, NT (Mannosyl-oligosaccharide Glucosidase, Processing A-glucosidase I, MOGS) with the following immunoassay(s):
Western Blot (WB) (Western blot analysis of anti-GCS1 Pab in 293 cell line lysates (35ug/lane). GCS1 (arrow) was detected using the purified Pab.)
GCS1 cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.
Immunogen: Synthetic peptide selected from the N-terminal region of human GCS1 (KLH). In general, we may offer more than one antibody to a given target to enable options for the researcher. Available antibodies recognizing gcs1 are readily searchable from our website. Different antibodies against the same target such as gcs1 may be optimized or tested for different applications and species. This enables researchers to select the option that may be best for their model system, to screen more than antibody to determine which one may be best for their model system, as well as to use more than one antibody to follow up on and validate their results. The following patways have been known to be associated with this gene.