GALE Antibody - N-terminal regio : HRP (ARP54393_P050-HRP)

UDP-galactose-4-epimerase

SDR1E1

2582

Q14376

NP_001008217

Human, Mouse, Rat, Cow, Dog, Guinea Pig, Pig, Rabbit

The immunogen is a synthetic peptide directed towards the N terminal region of human GALE

GALE is an UDP-galactose-4-epimerase which catalyzes two distinct but analogous reactions: the epimerization of UDP-glucose to UDP-galactose, and the epimerization of UDP-N-acetylglucosamine to UDP-N-acetylgalactosamine. The bifunctional nature of the enzyme has the important metabolic consequence that mutant cells (or individuals) are dependent not only on exogenous galactose, but also on exogenous N-acetylgalactosamine as a necessary precursor for the synthesis of glycoproteins and glycolipids. Mutations in this gene result in epimerase-deficiency galactosemia, also referred to as galactosemia type 3, a disease characterized by liver damage, early-onset cataracts, deafness and mental retardation, with symptoms ranging from mild ('peripheral' form) to severe ('generalized' form) . This gene encodes UDP-galactose-4-epimerase which catalyzes two distinct but analogous reactions: the epimerization of UDP-glucose to UDP-galactose, and the epimerization of UDP-N-acetylglucosamine to UDP-N-acetylgalactosamine. The bifunctional nature of the enzyme has the important metabolic consequence that mutant cells (or individuals) are dependent not only on exogenous galactose, but also on exogenous N-acetylgalactosamine as a necessary precursor for the synthesis of glycoproteins and glycolipids. Mutations in this gene result in epimerase-deficiency galactosemia, also referred to as galactosemia type 3, a disease characterized by liver damage, early-onset cataracts, deafness and mental retardation, with symptoms ranging from mild ('peripheral' form) to severe ('generalized' form) . Multiple alternatively spliced transcripts encoding the same protein have been identified.

GALE; SUMO2; UBC; BAG3; FN1; APP

HRP: Horseradish Peroxidase

Polyclonal Antibody

WB

Affinity Purified

0.5 mg/ml

Cow: 93%; Dog: 86%; Guinea Pig: 86%; Human: 100%; Mouse: 91%; Pig: 91%; Rabbit: 92%; Rat: 91%

Liquid. Purified antibody is supplied in high phosphate PBS, 100 mm phosphate, 150 mM NaCl, pH 7.6.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

38kDa

Chhay, J.S., (2008) J. Inherit. Metab. Dis. 31 (1), 108-116

Wet Ice

348

GALE

GALE

Rabbit

UDP-glucose 4-epimerase

NM_001008216

AEKVLVTGGAGYIGSHTVLELLEAGYLPVVIDNFHNAFRGGGSLPESLRR