Anti-GCS1 (C-term) Rabbit pAb

Antibody type:Primary antibody

Label:Unconjugated

Modification:

Isotype:Rabbit Ig

Host:Rabbit

Application:WB

Purify method:Affinity purified

Species:Human,Mouse

Gene Name:gcs1

Synonyms:Mannosyl-oligosaccharide glucosidase, Processing A-glucosidase I, MOGS, GCS1

Gene Synonyms:

Gene Full Name:

Gene Infomation: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.

Antigen: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.

Antigen Synonyms:

Clonality:Polyclonal antibody

Source:HUMAN

Reaction:

Form:Liquid

Tested Applications:

• Western blot (1:100 to 1:500)
• Immunofluorescence (1:50 to 1:400)
• Immunohistochemistry (1:200 to 1:500)
• Flow cytometry analysis (1:200 to 1:500)
• Enzyme-linked Immunosorbent Assay (1:100-1:5000)

Note: Users are strongly advised to determine the optimal dilution of antibody to use for their specific applications.

Clone:RB4963/4964

Dilution:WB::1:1000

Mole Mass:

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Gene Id:

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Unigene:

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Tissue specificity:

Storage:

Buffer condition: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.

Background:

Molar Function:

Western Blot: 

Stability: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

Storage: Store at 4°C for frequent use. Stored at -20°C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Notes: For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.