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FIGURE: 1 / 1
GCS1 Antibody (PA5-88726) in WB
Western blot analysis of GCS1 in extracts of various cell lines. Samples were incubated with GCS1 Polyclonal antibody (Product # PA5-88726) using a dilution of 1:3,000, followed by HRP Goat Anti-Rabbit IgG (H+L) at a dilution of 1:10,000. Lysates/proteins: 25 µg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 90s.
Product Details
PA5-88726
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
Recombinant fusion protein containing a sequence corresponding to amino acids 60-320 of human MOGS (NP_0062932)
Conjugate
Form
Liquid
Concentration
0.70 mg/mL
Purification
Affinity Chromatography
Storage buffer
PBS, pH 7.3, with 50% glycerol
Contains
0.01% thimerosal
Storage conditions
-20° C, Avoid Freeze/Thaw Cycles
Shipping conditions
Wet ice
RRID
AB_2805092
Product Specific Information
Immunogen sequence: RWVLAWYRAR RAVTLHSAPP VLPADSSSPA VAPDLFWGTY RPHVYFGMKT RSPKPLLTGL MWAQQGTTPG TPKLRHTCEQ GDGVGPYGWE FHDGLSFGRQ HIQDGALRLT TEFVKRPGGQ HGGDWSWRVT VEPQDSGTSA LPLVSLFFYV VTDGKEVLLP EVGAKGQLKF ISGHTSELGD FRFTLLPPTS PGDTAPKYGS YNVFWTSNPG LPLLTEMVKS RLNSWFQHRP PGAPPERYLG LPGSLKWEDR GPSGQGQGQF L; Positive Samples: U-87MG, LO2, BxPC-3, OVCAR-3, Mouse Brain, Mouse Liver, Mouse Pancreas, Mouse Testis, Rat Liver; Cellular Location: Endoplasmic reticulum membrane, Single-pass type II membrane protein
Target Information
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.
⚠WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm. For more information go to www.P65Warnings.ca.gov.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: Endoplasmic reticulum alpha-glucosidase I; ER glu I; Glucosidase 1; glucosidase I; Glycoprotein-processing glucosidase I; Mannosyl-oligosaccharide glucosidase; Processing A-glucosidase I
Gene Aliases: 1810017N02Rik; AI181835; CDG2B; CWH41; DER7; GCS1; MOGS
UniProt ID: (Human) Q13724, (Mouse) Q80UM7
Entrez Gene ID: (Human) 7841, (Mouse) 57377
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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