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Invitrogen™ GCS1 Polyclonal Antibody
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Catalog No. PIPA588726
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100 μL
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PIPA588726 100 μL
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Catalog No. PIPA588726 Supplier Invitrogen™ Supplier No. PA588726
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Description

Rabbit Polyclonal Antibody

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.

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.
TRUSTED_SUSTAINABILITY

Specifications

Antigen GCS1
Applications ELISA, Western Blot
Classification Polyclonal
Concentration 0.70 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.01% thimerosal; pH 7.3
Gene MOGS
Gene Accession No. Q13724, Q80UM7
Gene Alias 1810017N02Rik; AI181835; CDG2B; CWH41; DER7; GCS1; Glucosidase 1; glucosidase I; glycoprotein-processing glucosidase I; Mannosyl-oligosaccharide glucosidase; Mogs; Processing A-glucosidase I
Gene Symbols MOGS
Host Species Rabbit
Immunogen Recombinant fusion protein containing a sequence corresponding to amino acids 60-320 of human MOGS (NP_0062932).
Purification Method Affinity Chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 57377, 7841
Target Species Human, Mouse, Rat
Content And Storage -20°C, Avoid Freeze/Thaw Cycles
Product Type Antibody
Form Liquid
Isotype IgG
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