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GALE Antibody - N-terminal region : Biotin (ARP54393_P050-Biotin)

Product Specifications

Gene Name

UDP-galactose-4-epimerase

Gene Aliases

SDR1E1

Gene ID

2582

Swiss Prot

Q14376

Accession Number

NP_001008217

Host

Rabbit

Reactivity

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

Immunogen

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

Target

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.

Partner Proteins

GALE; SUMO2; UBC; BAG3; FN1; APP

Clonality

Polyclonal

Conjugation

Biotin

Type

Polyclonal Antibody

Applications

WB

Purification

Affinity Purified

Concentration

0.5 mg/ml

Homology

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

Format

Liquid. Purified antibody supplied in 1x PBS buffer.

Reconstitution

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.

Molecular Weight

38kDa

References & Citations

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

Shipping Conditions

Wet Ice

Product Datasheet

Anti-GALE (ARP54393_P050-Biotin) antibody

Protein Length

348

NCBI Gene Symbol

GALE

NCBI GB Accession Number

GALE

Host or Source

Rabbit

Protein Name

UDP-glucose 4-epimerase

Nucleotide Accession Number

NM_001008216

Peptide Sequence

AEKVLVTGGAGYIGSHTVLELLEAGYLPVVIDNFHNAFRGGGSLPESLRR

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