天门冬酰胺连接糖基化11抗体

ALG11 Antibody

Cat#：PL0306109

Isotype:Rabbit Polyclonal IgG

Immunogen：Peptide derived from human ALG11.

Purification: Immunogen affinity purified

Storage Buffer: PBS, pH 7.4(1%BSA and 0.1% Sodium azide)

Background：

Mannosyltransferase involved in the last steps of the synthesis of Man5GlcNAc(2)-PP-dolichol core oligosaccharide on the cytoplasmic face of the endoplasmic reticulum. Catalyzes the addition of the 4th and 5th mannose residues to the dolichol-linked oligosaccharide chain.Involvement in disease:Defects in ALG11 are the cause of congenital disorder of glycosylation type 1P (CDG1P). A multisystem disorder caused by a defect in glycoprotein biosynthesis and characterized by under-glycosylated serum glycoproteins. Congenital disorders of glycosylation result in a wide variety of clinical features, such as defects in the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency.

Specificity Reaction：Hu, Mo, Rat, Dog, Hor

Application Notes:WB: 1:100-500 E: 1:500-1000 IP: 1:20-100 IHC: 1:100-500 IF: 1:100-500

Storage ：

Store at –20ºC for one year. Avoid repeated freeze-thaw cycles. At 4ºC for 2-3 weeks after reconstituted.

This product for research use only, not for use in humans, therapeutic or diagnostic procedures.

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