GCS1 Antibody (N-term) Blocking Peptide
€363.00
In stock
SKU
AC-BP2315a
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.
Other Names:
Mannosyl-oligosaccharide glucosidase, Processing A-glucosidase I, MOGS, GCS1
Target/Specificity:
The synthetic peptide sequence used to generate the antibody AP2315a was selected from the N-term region of human GCS1 . A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
Gene Name: MOGS (HGNC:24862)
Gene ID: 7841
Primary Accession: Q13724
Other Accession: NP_006293
Format: Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.
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.
Other Names:
Mannosyl-oligosaccharide glucosidase, Processing A-glucosidase I, MOGS, GCS1
Target/Specificity:
The synthetic peptide sequence used to generate the antibody AP2315a was selected from the N-term region of human GCS1 . A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
Gene Name: MOGS (HGNC:24862)
Gene ID: 7841
Primary Accession: Q13724
Other Accession: NP_006293
Format: Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.
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