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  6. GCS1 Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech

GCS1 Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech

Rabbit Polyclonal Antibody

1710.33 NOK - 4836.10 NOK

Specifications

Antigen GCS1
Concentration 0.13 mg/mL
Applications Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Conjugate Unconjugated
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Products 2
Product Code Brand Quantity Price Quantity & Availability  
Product Code Brand Quantity Price Quantity & Availability  
16857854
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Proteintech
17859-1-AP-20UL 		20 μL
1710.33 NOK
20µL
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16847854
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Proteintech
17859-1-AP-150UL 		150 μL
4836.10 NOK
150µL
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Description

Description

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.
Specifications

Specifications

GCS1
Immunohistochemistry (Paraffin), Western Blot
Unconjugated
Rabbit
Rat, Mouse, Human
O88941, Q13724, Q80UM7
57377, 7841, 78947
GCS1 Fusion Protein Ag12321
Primary
-20°C
MOGS
0.13 mg/mL
Polyclonal
Liquid
RUO
PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
GCS1, MOGS, Processing A glucosidase I
Mogs
IgG
Antigen Affinity Chromatography
Antibody
Videos
SDS
Documents

Documents

Product Certifications
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