Name: Anti-MOGS Antibody (CAB12725)
Brand: Cell Biology Antibodies 3
SKU: CAB12725
Availability: OutOfStock

Antibody Name:	Anti-MOGS Antibody
Antibody SKU:	CAB12725
Antibody Size:	20uL, 50uL, 100uL
Application:	WB
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 60-320 of human MOGS (NP_006293.2).

Application:	WB
Recommended Dilution:	WB 1:500 - 1:2000
Reactivity:	Human, Mouse, Rat
Positive Samples:	U-87MG, LO2, BxPC-3, OVCAR-3, Mouse Brain, Mouse Liver, Mouse Pancreas, Mouse Testis, Rat Liver

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 60-320 of human MOGS (NP_006293.2).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	RWVL AWYR ARRA VTLH SAPP VLPA DSSS PAVA PDLF WGTY RPHV YFGM KTRS PKPL LTGL MWAQ QGTT PGTP KLRH TCEQ GDGV GPYG WEFH DGLS FGRQ HIQD GALR LTTE FVKR PGGQ HGGD WSWR VTVE PQDS GTSA LPLV SLFF YVVT DGKE VLLP EVGA KGQL KFIS GHTS ELGD FRFT LLPP TSPG DTAP KYGS YNVF WTSN PGLP LLTE MVKS RLNS WFQH RPPG APPE RYLG LPGS LKWE DRGP SGQG QGQF L
Gene ID:	7841
Uniprot:	Q13724
Cellular Location:	Endoplasmic reticulum membrane, Single-pass type II membrane protein
Calculated MW:	80kDa/91kDa
Observed MW:	80kDa

Synonyms:

MOGS, CDG2B, CWH41, DER7, GCS1

Background:

This gene encodes the first enzyme in the N-linked oligosaccharide processing pathway. The enzyme cleaves the distal alpha-1, 2-linked glucose residue from the Glc(3)-Man(9)-GlcNAc(2) oligosaccharide precursor. This protein is located in the lumen of the endoplasmic reticulum. Defects in this gene are a cause of type IIb congenital disorder of glycosylation (CDGIIb). Two transcript variants encoding different isoforms have been found for this gene.

UniProt Protein Function:	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 MOGS are the cause of type IIb congenital disorder of glycosylation (CDGIIb); also known as glucosidase I deficiency. CDGIIb 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 of the infant included hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course was progressive and the infant did not survive more than a few months. Belongs to the glycosyl hydrolase 63 family.
UniProt Protein Details:	Protein type:EC 3.2.1.106; Endoplasmic reticulum; Glycan Metabolism - N-glycan biosynthesis; Hydrolase; Membrane protein, integral Chromosomal Location of Human Ortholog: 2p13.1 Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane; membrane Molecular Function:glucosidase activity; mannosyl-oligosaccharide glucosidase activity Biological Process: protein amino acid N-linked glycosylation; protein folding Disease: Congenital Disorder Of Glycosylation, Type Iib
NCBI Summary:	This gene encodes the first enzyme in the N-linked oligosaccharide processing pathway. The enzyme cleaves the distal alpha-1,2-linked glucose residue from the Glc(3)-Man(9)-GlcNAc(2) oligosaccharide precursor. This protein is located in the lumen of the endoplasmic reticulum. Defects in this gene are a cause of type IIb congenital disorder of glycosylation (CDGIIb). Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2009]
UniProt Code:	Q13724
NCBI GenInfo Identifier:	116242490
NCBI Gene ID:	7841
NCBI Accession:	Q13724.5
UniProt Secondary Accession:	Q13724,Q17RN9, Q8TCT5, A8K938, F5H6D0,
UniProt Related Accession:	Q13724
Molecular Weight:	92kDa
NCBI Full Name:	Mannosyl-oligosaccharide glucosidase
NCBI Synonym Full Names:	mannosyl-oligosaccharide glucosidase
NCBI Official Symbol:	MOGS
NCBI Official Synonym Symbols:	DER7; GCS1; CDG2B; CWH41
NCBI Protein Information:	mannosyl-oligosaccharide glucosidase
UniProt Protein Name:	Mannosyl-oligosaccharide glucosidase
UniProt Synonym Protein Names:	Processing A-glucosidase I
Protein Family:	Mannosyl-oligosaccharide glucosidase
UniProt Gene Name:	MOGS

Western blot analysis of extracts of various cell lines, using MOGS antibody (CAB12725) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.

Antibodies

MOGS Antibody (PACO10545)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)