Product Description

ALG1 Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES18398-50, ES18398-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

Background: The enzyme encoded by this gene catalyzes the first mannosylation step in the biosynthesis of lipid-linked oligosaccharides. This gene is mutated in congenital disorder of glycosylation type Ik. [provided by RefSeq, Dec 2008],

Reactivity: Human, Mouse

Host: Rabbit

Applications: WB

Clonality: Polyclonal

Immunogen: Synthesized peptide derived from human ALG1 AA range: 180-230

Isotype: IgG

Target: ALG1

Specificity: This antibody detects endogenous levels of ALG1 at Human/Mouse

Dilution: WB 1：500-2000

Purification: The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.

Concentration: 1 mg/ml,

Formlation: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Molecular Weight (Da): 51kD

Fields: N-Glycan biosynthesis, Various types of N-glycan biosynthesis, Metabolic pathways

Function: Catalytic Activity: GDP-mannose + chitobiosyldiphosphodolichol = GDP + beta-1,4-D-mannosylchitobiosyldiphosphodolichol
Disease: Defects in ALG1 are the cause of congenital disorder of glycosylation type 1K (CDG1K) [MIM: 608540]. CDGs are a family of severe inherited diseases caused by a defect in protein N-glycosylation. They are characterized by under-glycosylated serum proteins. These multisystem disorders present with a wide variety of clinical features, such as disorders of the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions
Function: Participates in the formation of the lipid-linked precursor oligosaccharide for N-glycosylation. Involved in assembli

Subcellular Location: Endoplasmic reticulum membrane, Single-pass type II membrane protein

Storage and Stability: -20°C/1 year

Gene Name: ALG1 HMAT1 HMT1 PSEC0061 UNQ861/PRO1870

Protein Name: ALG1

Mouse Gene ID: 208211

Mouse SwissProt Number: Q921Q3

Research Use Only