Anti-GCS1 (N-term) Antibody

Product Description

Specifications

Size	400 µL
Estimated Lead Time	1-2 weeks
Species	Human
Host Species	Rabbit
Accession Number	Q13724\|NP_006293
Gene Id	7841
Gene Symbols	MOGS
Protein Name / Synonyms	Mannosyl-oligosaccharide glucosidase, Processing A-glucosidase I, MOGS, GCS1
Clonality	Polyclonal
Clone	RB4961/4962
Isotype	Kappa, Rabbit
Recommended Applications	Western Blotting
Shipping Type	Blue ice
Storage	-20°C

Description

Introduction

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 Information

NCBI Accession #

NP_001139630.1;NP_006293.2

Antigen Type

Synthetic Peptide

Format

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Calculated Molecular Weight (Da)

91918

Antigen Source

Human

Images

WB (1:1000)

Western blot analysis of anti-GCS1 Pab (Cat. #102-25379) in 293 cell line lysates (35ug/lane). GCS1 (arrow) was detected using the purified Pab.

Storage/Stability

2-8°C (short-term); -20°C (long-term)

Expiration:

12 months from the date of shipment when stored properly.

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