GCS1 Antibody (C-term)

日期：2020-08-01 00:36　人气：

Catalog_no	AB2066
Product_name	GCS1 Antibody (C-term)
Category	抗原抗体
Reactivity	H, M
Size	100μL/50μL
Price	2000.00/1100.00
immunogen	HUMAN:796-826
Specificity	This GCS1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 796-826 amino acids from the C-terminal region of human GCS1.
Dilution	WB,1:1000;
Purification	Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.
othername	Mannosyl-oligosaccharide glucosidase, Processing A-glucosidase I, MOGS, GCS1
Isotype	Rabbit Ig
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.
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