Rabbit anti-Human GCS1 Polyclonal Antibody | anti-gcs1 antibody

GCS1, NT (Mannosyl-oligosaccharide Glucosidase, Processing A-glucosidase I, MOGS)

Cat. Num. AAA624030

• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Affinity Purified; Purified by Protein G affinity chromatography.
• Synonyms: GCS1; Polyclonal Antibody; NT (Mannosyl-oligosaccharide Glucosidase; Processing A-glucosidase I; MOGS); Anti -GCS1; anti-gcs1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human GCS1.
• Purity/Purification: Affinity Purified; Purified by Protein G affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, 0.09% sodium azide.

• Applicable Applications for anti-gcs1 antibody: ELISA (EL/EIA), Western Blot (WB)
• Application Notes: Suitable for use in ELISA and Western Blot.; Dilution: ELISA: 1:1,000; Western Blot: 1:100-1:500
• Immunogen: Synthetic peptide selected from the N-terminal region of human GCS1 (KLH).
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western blot analysis of anti-GCS1 Pab in 293 cell line lysates (35ug/lane). GCS1 (arrow) was detected using the purified Pab.)

Related Product Information for anti-gcs1 antibody

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.

Product Categories/Family for anti-gcs1 antibody

Antibodies; Abs to Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 19113943
• NCBI GeneID: 2541881
• NCBI Accession #: NP_593031.1
• NCBI GenBank Nucleotide #: NM_001018430.1
• UniProt Accession #: Q09768
• Molecular Weight: 76,523 Da
• NCBI Official Full Name: glutamate-cysteine ligase Gcs1
• NCBI Official Symbol: gcs1
• NCBI Protein Information: glutamate-cysteine ligase Gcs1
• UniProt Protein Name: Glutamate--cysteine ligase
• Protein Family: Mannosyl-oligosaccharide glucosidase
• UniProt Gene Name: gcs1
• UniProt Entry Name: GSH1_SCHPO

Uniprot Description

Catalytic activity: ATP + L-glutamate + L-cysteine = ADP + phosphate + gamma-L-glutamyl-L-cysteine.

Pathway: Sulfur metabolism; glutathione biosynthesis; glutathione from L-cysteine and L-glutamate: step 1/2.

Subunit structure: Heterodimer of a catalytic heavy chain and a regulatory light chain

Sequence similarities: Belongs to the glutamate--cysteine ligase type 3 family.