Products for Research Use Only
RegisterLogin

GCS1 Antibody

CAT: 0223-61-369Size: 400 µLDry Ice: NoHazardous: No
Product image 1
1 / 1
CAT#:0223-61-369Size:400 µL
Selected
24/48H Stock Items & 2 to 6 Weeks non Stock Items.
Quick Request Actions
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
WB
Protein ID
116242490
Physical Properties
Liquid

Related Products

CatalogName
15-155GCSH Antibody
58-828GCSH Antibody
33-341GCSF Antibody [SPM468]
64-160WAS Antibody
60-596WAS Antibody
13-349WAS Antibody
62-828Neprilysin Antibody
61-580CAPZB Antibody
55-580HCG_2024410 Antibody
30-580ACPT Antibody

Alternative Products

CatalogName
70R-6629GCS1 antibody
PAab03394Anti-GCS1 antibody
bs-13322RGCS1 Polyclonal Antibody
MBS9214258-01GCS1 Antibody (C-term)
NBP3-03774-100ulRabbit Polyclonal GCS1 Antibody
E28PN6310GCS1 Rabbit Polyclonal Antibody
SL13322R-01Rabbit Anti-GCS1 antibody
A08252-1Anti-GCS1/MOGS Antibody
GTR18970341GCS1 Antibody Blocking peptide
GTR18329295-01GCS1 Antibody (N-term)

Popular Products