50-198-2802
GCS1 Rabbit anti-Human, Polyclonal, Bioss
Manufacturer: Bioss
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| Pack Size | SKU | Availability | Price |
|---|---|---|---|
| Each of 1 | 50-198-2802-Each-of-1 | In Stock | ₹ 36,897.75 |
50-198-2802 - Each of 1
In Stock
Quantity
1
Base Price: ₹ 36,897.75
GST (18%): ₹ 6,641.595
Total Price: ₹ 43,539.345
Antigen
GCS1
Classification
Polyclonal
Conjugate
Unconjugated
Gene
MOGS
Gene Alias
1810017N02Rik; AI181835; CDG2B; CWH41; DER7; Gcs1; Glucosidase 1; glucosidase I; Glycoprotein-processing glucosidase I; Mannosyl-oligosaccharide glucosidase; Mogs; Processing A-glucosidase I
Host Species
Rabbit
Purification Method
Protein A
Regulatory Status
RUO
Gene ID (Entrez)
7841
Content And Storage
-20°C
Form
Liquid
Applications
Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
Concentration
1 μg/mL
Formulation
PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
Gene Accession No.
Q13724
Gene Symbols
MOGS
Immunogen
KLH conjugated synthetic peptide derived from human GCS1.
Quantity
100 μL
Primary or Secondary
Primary
Target Species
Human
Product Type
Antibody
Isotype
IgG
Description
- 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.
Compare Similar Items
Show Difference
Antigen: GCS1
Classification: Polyclonal
Conjugate: Unconjugated
Gene: MOGS
Gene Alias: 1810017N02Rik; AI181835; CDG2B; CWH41; DER7; Gcs1; Glucosidase 1; glucosidase I; Glycoprotein-processing glucosidase I; Mannosyl-oligosaccharide glucosidase; Mogs; Processing A-glucosidase I
Host Species: Rabbit
Purification Method: Protein A
Regulatory Status: RUO
Gene ID (Entrez): 7841
Content And Storage: -20°C
Form: Liquid
Applications: Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
Concentration: 1 μg/mL
Formulation: PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
Gene Accession No.: Q13724
Gene Symbols: MOGS
Immunogen: KLH conjugated synthetic peptide derived from human GCS1.
Quantity: 100 μL
Primary or Secondary: Primary
Target Species: Human
Product Type: Antibody
Isotype: IgG
Antigen:
GCS1
Classification:
Polyclonal
Conjugate:
Unconjugated
Gene:
MOGS
Gene Alias:
1810017N02Rik; AI181835; CDG2B; CWH41; DER7; Gcs1; Glucosidase 1; glucosidase I; Glycoprotein-processing glucosidase I; Mannosyl-oligosaccharide glucosidase; Mogs; Processing A-glucosidase I
Host Species:
Rabbit
Purification Method:
Protein A
Regulatory Status:
RUO
Gene ID (Entrez):
7841
Content And Storage:
-20°C
Form:
Liquid
Applications:
Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
Concentration:
1 μg/mL
Formulation:
PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
Gene Accession No.:
Q13724
Gene Symbols:
MOGS
Immunogen:
KLH conjugated synthetic peptide derived from human GCS1.
Quantity:
100 μL
Primary or Secondary:
Primary
Target Species:
Human
Product Type:
Antibody
Isotype:
IgG
Antigen: GDPD1/GDE4
Classification: Polyclonal
Conjugate: Unconjugated
Gene: Gdpd1
Gene Alias: 2610020H15Rik; BOS_18359; Gde4; GDPD1; glycerophosphodiester phosphodiesterase 1-like; Glycerophosphodiester phosphodiesterase 4; glycerophosphodiester phosphodiesterase domain containing 1; Glycerophosphodiester phosphodiesterase domain-containing protein 1; LOC100352793; Lysophospholipase D GDPD1; RGD1311813
Host Species: Rabbit
Purification Method: Protein A
Regulatory Status: RUO
Gene ID (Entrez): 284161
Content And Storage: -20°C
Form: Liquid
Applications: Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
Concentration: 1 μg/mL
Formulation: PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
Gene Accession No.: Q8N9F7
Gene Symbols: Gdpd1
Immunogen: KLH conjugated synthetic peptide derived from human GDPD1/GDE4.
Quantity: 100 μL
Primary or Secondary: Primary
Target Species: Human
Product Type: Antibody
Isotype: IgG
Antigen:
GDPD1/GDE4
Classification:
Polyclonal
Conjugate:
Unconjugated
Gene:
Gdpd1
Gene Alias:
2610020H15Rik; BOS_18359; Gde4; GDPD1; glycerophosphodiester phosphodiesterase 1-like; Glycerophosphodiester phosphodiesterase 4; glycerophosphodiester phosphodiesterase domain containing 1; Glycerophosphodiester phosphodiesterase domain-containing protein 1; LOC100352793; Lysophospholipase D GDPD1; RGD1311813
Host Species:
Rabbit
Purification Method:
Protein A
Regulatory Status:
RUO
Gene ID (Entrez):
284161
Content And Storage:
-20°C
Form:
Liquid
Applications:
Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
Concentration:
1 μg/mL
Formulation:
PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
Gene Accession No.:
Q8N9F7
Gene Symbols:
Gdpd1
Immunogen:
KLH conjugated synthetic peptide derived from human GDPD1/GDE4.
Quantity:
100 μL
Primary or Secondary:
Primary
Target Species:
Human
Product Type:
Antibody
Isotype:
IgG
Antigen: GDPGP1/C15orf58
Classification: Polyclonal
Conjugate: Unconjugated
Gene: GDPGP1
Gene Alias: C15orf58; D330012F22Rik; GDP-D-glucose phosphorylase 1; GDP-D-glucose phosphorylase C15orf58; GDP-D-glucose phosphorylase C15orf58 homolog; GDPGP1; VTC2
Host Species: Rabbit
Purification Method: Protein A
Regulatory Status: RUO
Gene ID (Entrez): 390637
Content And Storage: -20°C
Form: Liquid
Applications: Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
Concentration: 1 μg/mL
Formulation: PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
Gene Accession No.: Q6ZNW5
Gene Symbols: GDPGP1
Immunogen: KLH conjugated synthetic peptide derived from human GDPGP1/C15orf58.
Quantity: 100 μL
Primary or Secondary: Primary
Target Species: Human
Product Type: Antibody
Isotype: IgG
Antigen:
GDPGP1/C15orf58
Classification:
Polyclonal
Conjugate:
Unconjugated
Gene:
GDPGP1
Gene Alias:
C15orf58; D330012F22Rik; GDP-D-glucose phosphorylase 1; GDP-D-glucose phosphorylase C15orf58; GDP-D-glucose phosphorylase C15orf58 homolog; GDPGP1; VTC2
Host Species:
Rabbit
Purification Method:
Protein A
Regulatory Status:
RUO
Gene ID (Entrez):
390637
Content And Storage:
-20°C
Form:
Liquid
Applications:
Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
Concentration:
1 μg/mL
Formulation:
PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
Gene Accession No.:
Q6ZNW5
Gene Symbols:
GDPGP1
Immunogen:
KLH conjugated synthetic peptide derived from human GDPGP1/C15orf58.
Quantity:
100 μL
Primary or Secondary:
Primary
Target Species:
Human
Product Type:
Antibody
Isotype:
IgG
Antigen: Gemin 4
Classification: Polyclonal
Conjugate: Unconjugated
Gene: GEMIN4
Gene Alias: 4932415L08Rik; AU015359; BOS_18587; Component of gems 4; DKFZp434B131; DKFZp434D174; gem (nuclear organelle) associated protein 4; gem nuclear organelle associated protein 4; Gem-associated protein 4; GEMIN4; Gemin-4; gemin4-like; HC56; HCAP1; HCC-associated protein 1; HHRF-1; LOC100345108; p97; RGD1563715
Host Species: Rabbit
Purification Method: Protein A
Regulatory Status: RUO
Gene ID (Entrez): 50628
Content And Storage: -20°C
Form: Liquid
Applications: Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
Concentration: 1 μg/mL
Formulation: PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
Gene Accession No.: P57678
Gene Symbols: GEMIN4
Immunogen: KLH conjugated synthetic peptide derived from human Gemin 4.
Quantity: 100 μL
Primary or Secondary: Primary
Target Species: Human
Product Type: Antibody
Isotype: IgG
Antigen:
Gemin 4
Classification:
Polyclonal
Conjugate:
Unconjugated
Gene:
GEMIN4
Gene Alias:
4932415L08Rik; AU015359; BOS_18587; Component of gems 4; DKFZp434B131; DKFZp434D174; gem (nuclear organelle) associated protein 4; gem nuclear organelle associated protein 4; Gem-associated protein 4; GEMIN4; Gemin-4; gemin4-like; HC56; HCAP1; HCC-associated protein 1; HHRF-1; LOC100345108; p97; RGD1563715
Host Species:
Rabbit
Purification Method:
Protein A
Regulatory Status:
RUO
Gene ID (Entrez):
50628
Content And Storage:
-20°C
Form:
Liquid
Applications:
Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
Concentration:
1 μg/mL
Formulation:
PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
Gene Accession No.:
P57678
Gene Symbols:
GEMIN4
Immunogen:
KLH conjugated synthetic peptide derived from human Gemin 4.
Quantity:
100 μL
Primary or Secondary:
Primary
Target Species:
Human
Product Type:
Antibody
Isotype:
IgG