Western Blot (WB)
(GNPTG Antibody (C-term) (MBS9206936) western blot analysis in NCI-H292 cell line lysates (35ug/lane).This demonstrates the GNPTG antibody detected the GNPTG protein (arrow).)
Rabbit anti-Human GNPTG Polyclonal Antibody | anti-GNPTG antibody
GNPTG Antibody (C-term)
Western Blot (WB)
(GNPTG Antibody (C-term) (MBS9206936) western blot analysis in NCI-H292 cell line lysates (35ug/lane).This demonstrates the GNPTG antibody detected the GNPTG protein (arrow).)
Western Blot (WB)
(GNPTG Antibody (C-term) (MBS9206936) western blot analysis in NCI-H292 cell line lysates (35ug/lane).This demonstrates the GNPTG antibody detected the GNPTG protein (arrow).)
N-acetylglucosamine-1-phosphotransferase complex. This hexameric
complex, composed of alpha, beta and gamma subunits, catalyzes the
first step in synthesis of a mannose 6-phosphate lysosomal
recognition marker. This enzyme complex is necessary for targeting
of lysosomal hydrolases to the lysosome. Mutations in the gene
encoding the gamma subunit have been associated with mucolipidosis
IIIC, also known as mucolipidosis III gamma.
Qian, Y., et al. J. Biol. Chem. 285(5):3360-3370(2010)
Pohl, S., et al. Am. J. Med. Genet. A 152A (1), 124-132 (2010) :
Encarnacao, M., et al. Clin. Genet. 76(1):76-84(2009)
Persichetti, E., et al. Hum. Mutat. 30(6):978-984(2009)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes the gamma sunbunit of the N-acetylglucosamine-1-phosphotransferase complex. This hexameric complex, composed of alpha, beta and gamma subunits, catalyzes the first step in synthesis of a mannose 6-phosphate lysosomal recognition marker. This enzyme complex is necessary for targeting of lysosomal hydrolases to the lysosome. Mutations in the gene encoding the gamma subunit have been associated with mucolipidosis IIIC, also known as mucolipidosis III gamma.[provided by RefSeq, Feb 2010]
Uniprot Description
GNPTG: the gamma subunit of N-acetylglucosamine-1-phosphotransferase. May recognize the substrate of GlcNAc-1-phosphotransferase but also lysosomal proteins with mannose-6-phosphate residues. Defects in GNPTG cause a variant of pseudo-Hurler polydystrophy, an autosomal recessive disease of lysosomal hydrolase trafficking.
Protein type: Secreted, signal peptide; Secreted
Chromosomal Location of Human Ortholog: 16p13.3
Cellular Component: Golgi membrane; Golgi apparatus
Molecular Function: protein homodimerization activity; UDP-N-acetylglucosamine-lysosomal-enzyme N-acetylglucosaminephosphotransferase activity
Biological Process: N-glycan processing to lysosome; carbohydrate phosphorylation
Disease: Mucolipidosis Iii Gamma
Research Articles on GNPTG
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Product Notes
The GNPTG gnptg (Catalog #AAA9206936) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 266-296. The GNPTG Antibody (C-term) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's GNPTG can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB~~1:1000. Researchers should empirically determine the suitability of the GNPTG gnptg 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. It is sometimes possible for the material contained within the vial of "GNPTG, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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