GCS1 Antibody

Product Specifications

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.

NCBI Gene ID

7841

Swiss Prot

Q13724

Accession Number

Q13724

Host

Rabbit

Reactivity

Human, Mouse

Immunogen

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.

Clonality

Polyclonal

Conjugation

Unconjugated

Type

Primary Antibodies

Field of Research

Other

Purification

This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis

Concentration

Batch dependent

Buffer

Supplied in PBS with 0.09% (W/V) sodium azide.

Modification

None

Shipping Conditions

Blue Ice

Storage Conditions

Store at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

Calculated Molecular Weight

92 kDa

Fragment

Rabbit Ig

Applications Notes

For WB starting dilution is: 1:1000

Symbol

MOGS

NCBI Official Name

Mannosyl-oligosaccharide glucosidase

NCBI Organism

Homo sapiens

Background Reference 01

Volker, C., et al., Glycobiology 12 (8) :473-483 (2002) .

Background Reference 02

De Praeter, C.M., et al., Am. J. Hum. Genet. 66 (6) :1744-1756 (2000) .

Background Reference 03

Kalz-Fuller, B., et al., Eur. J. Biochem. 231 (2) :344-351 (1995) .

Background Reference 04

Kalz-Fueller, B., et al., Eur. J. Biochem. 249, 912-912 (1997) .

Other Product Names

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

Tested Applications

Protein ID

116242490

Physical Properties

Liquid

Curated Selection

Explore Other Products

Discover premium biology products from our extensive collection of 20M+ items

A11320-25 25 mg

BAY 61-3606 25mg

Ask

View Details

42075011 1.0 µg DNA

RPS12P30 ORF Vector (Human) (pORF)

Ask

View Details

GTR15407761 1 Vial

Human TRAK2 Knockout A549 cell line

Ask

View Details

38190084 1.0 µg

Ptprz1 3'UTR Lenti-reporter-Luc Vector

Ask

View Details

541457-01 30ul

Rab 33A discontinued

Ask

View Details

541457-02 100 µL

Rab 33A discontinued

Ask

View Details