GCS1 antibody (C-Term)
Quick Overview for GCS1 antibody (C-Term) (ABIN357670)
Target
See all GCS1 (MOGS) AntibodiesReactivity
Host
Clonality
Conjugate
Application
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Binding Specificity
- C-Term
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Specificity
- This antibody is specific to GCS1 (C-term).
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Purification
- Protein G Chromatography, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.
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Immunogen
- This antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected form the C-terminal region of human GCS1.
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Isotype
- Ig Fraction
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Application Notes
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ELISA: 1/1,000. Western Blot: 1/100-1/500.
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user. -
Restrictions
- For Research Use only
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Format
- Liquid
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Concentration
- 0.25 mg/mL
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Buffer
- PBS with 0.09 % (W/V) Sodium Azide as preservative.
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Preservative
- Sodium azide
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Precaution of Use
- This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Handling Advice
- Avoid repeated freezing and thawing.
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Storage
- 4 °C/-20 °C
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Storage Comment
- Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at-20 °C for longer.
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- GCS1 (MOGS) (Mannosyl-Oligosaccharide Glucosidase (MOGS))
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Alternative Name
- MOGS / GCS1
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Background
- 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.Synonyms: Mannosyl-oligosaccharide glucosidase, Processing A-glucosidase I
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Molecular Weight
- 91840 Da
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Gene ID
- 7841, 5874
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UniProt
- Q13724
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Pathways
- SARS-CoV-2 Protein Interactome
Target
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