GCS1 (MOGS) (N-term) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	WB
Recommend Dilution	ELISA: 1/1,000. Western Blot: 1/50-1/100.
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	This antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the N-terminal region of human GCS1.
Specificity	This antibody is specific to GCS1 (N-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	Entrez Gene 7841 Human UniProt ID Q13724
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
Reference Data

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