GCS1 (MOGS) (C-term) Rabbit Polyclonal Antibody
CAT#: AP12153PU-N
GCS1 (MOGS) (C-term) rabbit polyclonal antibody, Purified
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CNY 6,160.00
货期*
5周
规格
Specifications
Product Data | |
Applications | WB |
Recommend Dilution | ELISA: 1/1,000. Western Blot: 1/100-1/500. |
Reactivity | Human, Mouse |
Host | Rabbit |
Clonality | Polyclonal |
Immunogen | This antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected form the C-terminal region of human GCS1. |
Specificity | This antibody is specific to GCS1 (C-term). |
Formulation | PBS with 0.09% (W/V) Sodium Azide as preservative. State: Purified State: Liquid purified Ig fraction. |
Concentration | lot specific |
Purification | Protein G Chromatography, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS. |
Conjugation | Unconjugated |
Storage Condition | Store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing. |
Gene Name | mannosyl-oligosaccharide glucosidase |
Database Link | |
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 | GCS1 |
Note | Predicted Molecular weight: 91840 Da |
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